Clinical Vignettes
A CASE OF ACYCLOVIR-INDUCED RESPIRATORY DEPRESSION IN PATIENT WITH END-STAGE RENAL
DISEASE
W. Hester
1; V.T. Martin1; S. Bansil1; C.J. Fichtenbaum1. 1University of Cincinnati, Cincinnati,
OH. (Tracking ID #115632)
LEARNING OBJECTIVES
1. Recognize the clinical setting of acyclovir-induced neurotoxicity. 2. Diagnose
and manage acyclovir-induced neuroxicity.
CASE
A 46 year-old woman with AIDS (recent CD 4 + lymphocyte count 145 per mL & plasma
HIV RNA level <400 copies per mL) and ESRD on continuous ambulatory peritoneal dialysis
(CAPD) presented with pain in her right thigh for three months. Doppler ultrasounds
were negative for deep venous thrombosis on two different tests. She had been in stable
health without recent opportunistic infections. Her past medical history was significant
for a history of shingles, orolabial herpes simplex disease, pancreatitis secondary
to nucleosides, Candida esophagitis, and asthma. Her physical exam revealed an edematous
right thigh and a tender 3 × 5 cm irregular ecchymotic area present on her lateral
thigh near a small shallow ulcer. A small vesicle has preceded the ulcer. Laboratory
data included a white blood cell count of 8.1K with 74% neutrophils; blood urea nitrogen
of 74 mg/dL; creatinine of 15.7 mg/dL; and creatinine kinase 1,303 U/L. The Alveolar-arterial
gradient was 71053.25 mmHg. Computerized Tomography of the right lower extremity with
contrast and multiplanar 3D reconstructions revealed no abscess. Empiric therapy with
intravenous acyclovir 5 mg/kg/day was empirically started on hospital day 2. The next
day the patient developed delirium and hypoxemia. The arterial blood gas revealed
a pH 7.21, PaCO2 67 mmHg, PaO2 163, HCO3 27 mmol/L, O2 saturation 97.8% on 100% oxygen
via a non-rebreathable mask. Chest radiography demonstrated pulmonary edema. Cultures
of the peritoneal fluid, bronchoalveolar lavage, blood, and spinal fluid, were all
negative. The skin biopsy demonstrated findings consistent with calciphylaxis and
pressure necrosis and the absence of viral inclusions. Acyclovir was discontinued
on the sixth hospital day and a serum acyclovir level 12 hours after stopping the
acyclovir was 5.5 mcg/mL (reported therapeutic peak range of 0.40–2.0 mcg/mL). Twenty-four
hours after stopping the acyclovir the patient became alert and was extubated within
48 hours. Given all of these findings, the patient was diagnosed with acyclovir-induced
respiratory depression.
DISCUSSION
Neurotoxicities such as lethargy, confusion, and delirium have been reported with
acyclovir and seem to be more prevalent in the setting of kidney dysfunction, but
have been identified in otherwise healthy individuals. To our knowledge, this is the
first case report of acyclovir leading to respiratory failure in a patient with chronic
renal disease. As demonstrated in this case, acyclovir should be used cautiously in
those with renal failure to prevent neurotoxicities.
A CASE OF AMIODARONE-INDUCED THYROTOXICOSIS
J.E. Adams
1. 1University of California, San Francisco, San Francisco, CA. (Tracking ID #115759)
LEARNING OBJECTIVES
1. Review Amiodarone's effects on thyroid function. 2. Diagnose and treat thyrotoxic
effects of Amiodarone.
CASE
62 y/o male presented to his primary medical doctor complaining of a several month
history of weakness, fatigue, hand tremor, and a ten pound weight loss. The patient
was started on Amiodarone 2 years ago for paroxysmal atrial fibrillation and had remained
in sinus rhythm without further complications. Upon initial work-up patient was found
to have an undetectable TSH, and an elevated free T4.
DISCUSSION
Up to 20% of patients on long-term therapy will develop hypothyroidism as a result
of toxic effects of Amiodarone, and 3% will develop hyperthyroidism. Hypothyroidism
occurs by several mechanisms, the most common being a destructive thyroiditis which
is often preceded by a hyperthyroid phase. Additionally, Amiodarone decreases the
peripheral conversion of T4 to T3 and acts to directly block the T3 receptor. Lastly,
synthesis of thyroid hormone is inhibited by high levels of iodine in Amiodarone (Wolff-Chaikoff
effect). Treatment of hypothyroidism is with replacement therapy and is rarely an
indication to discontinue therapy. Hyperthyroidism secondary to Amiodarone toxicity
also occurs by a variety of mechanisms. In Type 1, synthesis of T4 is increased due
to iodine load in a patient with underlying autonomy secondary to a nodule or goiter.
In Type 2, patients develop a destructive thyroiditis often followed by hypothyroidism.
Clinically, determining the mechanism of hyperthyroidism can be challenging but can
direct therapy. Detectable uptake on thyroid scan or nodules on exam suggest Type
1. Patients with Type 2 sometimes have elevated IL-6 levels. Doppler sonography to
assess vascularity and diagnose small nodules is successful in classifying 80% of
cases. Type 1 disease is treated with anti-thyroid drugs such as Methimazole and response
may be slow. Patients with Type 2 are treated with steroids and often respond quickly.
In clinical practice, patients are often treated with both, with the rapidity of response
guiding further treatment. In considering stopping therapy, it is important to weigh
the risks of chronic hyperthyroidism against the risk of arrhythmia. Amiodarone has
a very long half-life which prevents any immediate benefit in stopping the drug, and
symptoms may actually be exacerbated when the beta-blocking effects of Amiodarone
are lost. In general, thyrotoxicity is not an absolute contraindication for continuation
of Amiodarone and risks and benefits must be weighed carefully. In monitoring patients
on long-term therapy, TSH and FT4 should be followed every six months.
A CASE OF AMNESIA RESPONSIVE TO PHLEBOTOMY
H.A. Younes
1; R. Parker1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116839)
LEARNING OBJECTIVES
1) To diagnose and recognize the different kinds of erythrocytosis and polycythemia,
2) To learn about the complications of erythrocytosis, 3) To learn about the treatment
options of different kinds of erythrocytosis.
CASE
A 63 y.o. gentleman, with a past medical history significant for CAD, hypertension,
and recurrent DVT's, presented to his PCP office complaining of frequent forgetfulness
for 2 weeks. He reported the problem starting while he was on a plane trip from Denver
to Pittsburgh where he felt some shortness of breath with mild headache. When he reached
Pittsburgh, he couldn't tell where he came from, or the time of the day. He was even
somewhat confused about his personal belongings. This episode was followed with several
incidences of forgetfulness in his daily tasks, such as forgetting the water tap was
open and forgetting his daily schedule of activities. Physical exam revealed no localized
neurological deficit. An MRI of brain showed no evidence of a new ischemic or hemorrhagic
stroke, although it showed an old right MCA occipital lobe infarct. A hypercoagulable
workup was negative. A CBC showed a Hgb of 18 g/dL, a Hct of 54.3, and an RBC mass
of 5.83 × 10 to power 12 per L. WBC count was 6.6, and platelet count 147,000. A repeat
CBC confirmed above values. Serum viscosity and erythropoitin level proved normal.
A blood volume study showed normal RBC volume, with a low plasma volume, and a low
normal total blood volume; all findings consistent with a relative polycythemia. In
view of the persistent neurological findings, therapeutic phlebotomy of the patient
was done, with 500 ml removed each time. After a few sessions, the patient reported
his symptoms improving significantly, with a decrease in his hemoglobin and hematocrit
levels.
DISCUSSION
As opposed to absolute polycythemia (polycythemia vera or PV) where there is an absolute
increase in red cell mass, ‘relative’, ‘stress’, or ‘apparent’ polycythemia is defined
as an increase in hematocrit with normal red cell mass. Approximately 25% of cases
have reduced plasma volume. Possible causes of this relative polycythemia include
dehydration, alcohol, smoking, obesity, hypoxia, acute MI, and hypertension. PV is
known to cause both microvascular disturbances, such as peripheral ischemia and atypical
cerebral ischemic attacks, as well as major arterial and venous thromboembolism. However,
the risk of vascular occlusive episodes in relative polycythemia is not well known.
A study by Schwartz et al. comparing relative polycythemia with PV revealed significantly
more thromboembolic events (DVT/PE) in PV, but equal risk of cardiac and cerebrovascular
events. The fact that our patient's symptoms improved after phlebotomy as his hematocrit
decreased, suggests that his symptoms were atypical cerebral attacks. Although phlebotomy
is not considered a typical treatment modality of relative polycythemia, it worked
well with our patient.
A CASE OF CAMPYLOBACTER FETUS MENINGITIS IN A FORTY-YEAR OLD MAN
M.S. Divakaruni1; A. Hwang
2. 1Stanford University, Palo Alto, CA; 2Santa Clara Valley Medical Center, San Jose,
CA. (Tracking ID #116215)
LEARNING OBJECTIVES
1. Recognize Camplyobacter species as a potential etiology of bacterial meningitis
in patients with predisposing illness, including recent or distant neurosurgery, or
alcohol abuse. 2. Treat CNS infections with C. fetus with carbapenems or a third-generation
cephalosporin and an aminoglycoside.
CASE
Campylobacter is an uncommon cause of bacterial meningitis in adults. We report the
case of a 40-year old Vietnamese gentleman with a prior history of partial craniotomy
and alcohol abuse who was admitted with headache, fever, neck pain, and weight loss
over the preceding two weeks. The patient had reported a history of a flu-like illness
preceded by one day of non-bloody diarrhea, but these symptoms had resolved several
days prior to admission. On admission, he was febrile to 39.4 degrees centigrade and
had prominent nuchal rigidity and positive Kernig's and Brudzinski's signs, but an
otherwise normal neurological exam except for marked confusion. Laboratory data showed
serum WBC 15,000/mm3 with a left shift. Cerebrospinal fluid analysis showed 543 WBC/mm3
with 85% neutrophils and 15% lymphocytes, glucose of 24 mg/dl, and protein of 117
mg/dl. The fluid was India ink negative, cryptococcal antigen negative, and acid-fast
bacilli negative. The patient was initially treated with intravenous vancomycin and
cetriaxione. On the third hospital day, one out of four blood cultures began to grow
out gram-negative rods. The subsequent day, the patient's cerebrospinal fluid grew
out comma-shaped gram-negative rods suspicious for Campylobacter species. Vancomycin
was discontinued, and the patient was started on gentamicin in addition to ceftriaxone.
The patient responded rapidly to antibiotic therapy. Both the patient's blood and
cerebrospinal fluid cultures eventually returned with a final result of Campylobacter
fetus species. The patient was treated with a total of five days of parenteral gentamicin,
fifteen days of parenteral ceftriaxone, and an additional seven days of oral ciprofloxacin
for a total antibiotic course of twenty-one days. At the time of discharge the patient
was doing well, and had no further gastointestinal or neurologic symptoms.
DISCUSSION
In this case of Campylobacter fetus meningitis, the patient had predominately extra-intestinal
manifestations as is normally seen with C. fetus species, though with a one-day history
of diarrheal illness not usually reported with the organism. The patient had a predisposing
immunosuppressed state secondary to his alcohol abuse, as well as a prior history
of neurosurgery, consistent with previously reported cases. Given the incidence of
mortality reported in the case literature, and this patient's rapid response to the
selected antibiotic regimen, the early and appropriate treatment of C. fetus meningitis
appears to be clinically important.
A CASE OF HERPES ZOSTER ENCEPAHLITIS
S. Ramamurthy
1; M. Graham2. 1Medical College of Wisconsin, Germantown, WI; 2Medical College of
Wisconsin, Milwaukee, WI. (Tracking ID #115575)
LEARNING OBJECTIVES
Viral pathogens can cause a variety of syndromes when affecting the central nervous
system including aseptic meningitis and encephalitis.Varicella zoster virus (VZV)
is a rare cause of central nervous system syndromes. We discuss a patient who initially
presented with dermatomal zoster whose clinical course was complicated by the development
of VZV encephalitis with complications both from the primary disease process and the
appropriate therapy.
CASE
66 yr old Caucasian female with history of rheumatoid arthritis treated with methotrexate
who presented with mental status changes. Seven days prior to admission she developed
an erythematous rash on back and chest confined to right side of thorax. She was treated
with valacyclovir for two days and complained of pain at the site for which she was
prescribed vicodin and amitryptyline. The next day she was noted to be disoriented
by family and brought to ER. Physical exam revealed an erythematous vesicular rash
on her chest and back confined to the T2–T3 dermatome on right side. Neurological
exam was within normal limits except that she had difficulty finding words. CT scan
of the head on admission was normal and her labs were significant for hyponatremia
(123 mmol/l). Urine osmolality was 708 mosm/kg and serum osmolality was 267 mosm/kg
consistent with SIADH. She was placed on intravenous (IV) acyclovir and fluid restriction
for SIADH. Cerebrospinal fluid (CSF) analysis revealed an elevated white blood cell
count (321/cmm) with lymphocytic predominanace (90%) and elevated protein level (115
mg/dL). CSFanalysis for VZV by PCR was positive. On day 3 her creatinine level increased
and urinalysis revealed numerous crystals consistent with acyclovir induced nephropathy.
The acyclovir dose was adjusted based on renal function and she was given IV fluids.
On day 4 she complained of hallucinations and double vision. MRI of the head was normal.
She continued to improve with IV acyclovir and hyponatremia resolved. A repeat CSF
analysis was negative for VZV by PCR. Patient completed a two week course of IV acyclovir
and was discharged on oral valacyclovir for an additional week and neurontin for pain.
DISCUSSION
Herpes zoster encephalitis is rare and very few cases have been reported.We postulate
that in this case her immunosuppresed state on methotrexate was the main predisposing
factor. This case also highlights the complications of disease process, specifically
hyponatremia (SIADH) and adverse effect of treatment (acyclovir induced nephropathy)
and how to manage them astutely.
A CASE OF INTERNAL MAMMARY ARTERY STEAL SYNDROME
H.L. Korlakunta
1; D. Lakkireddy1; N. Mehta1; T. Lanspa1; I. Khan1. 1Creighton University, Omaha,
NE. (Tracking ID #115718)
LEARNING OBJECTIVES
To report a case of a patient with IMA steal syndrome after a LIMA bypass grafting
to the LAD who was successfully treated with percutaneous transcatheter endovascular
coiling of the anomalous lateral internal thoracic artery.
CASE
A 53-year male presented for evaluation of recurrent exertional angina more so with
upper body exercise. He had known diabetes, hypertension, hyperlipidemia, paroxysmal
atrial fibrillation and coronary artery disease with a 3-vessel coronary artery bypass
surgery done 4 years prior to presentation. A transradial coronary angiogram revealed
total occlusion of the RCA graft with 99% stenosis of mid RCA, which was successfully
treated with angioplasty and stent placement. The LCX graft was patent, LIMA was patent
but appeared to be a small vessel. There was 80% stenosis of mid LAD just proximal
to the LIMA insertion. During LIMA injection a parallel branch running lateral to
the LIMA graft was seen giving rise to anterior intercostals and perforating branches.
This fits the anatomic description of an anomalous IMA with a lateral internal mammary
artery. Patient then underwent an adenosine cardiolyte stress perfusion imaging which
showed mild to moderate reversible ischemia in the antero-septal and anterior walls.
Patient was started on a long acting nitrate in addition to his regular dose of beta
blockers, diuretic, angiotensin converting enzyme inhibitor and was advised to abstain
from upper body exertional activities. He was brought back a month later and a selective
catheterization followed by a coil embolization of the lateral internal thoracic artery
was performed with successful closure. There was a dramatic improvement to the flow
through the LIMA graft after closing the lateral branch. An exercise stress was performed
with no ischemic symptoms or EKG manifestations.
DISCUSSION
The internal mammary artery (IMA) is a conduit of choice for myocardial revascularization,
especially when the target vessel is the left anterior descending artery (LAD). Occasionally
IMA hypo perfusion occurs when there is inadequate flow through the IMA graft to the
LAD artery. The graft hypo perfusion can occur both acutely and chronically resulting
in Malperfusion Syndrome and Dysfunctional Graft with persistent ischemia in the region
of supply. This is a case of symptomatic LAD ischemia from a hypoperfusing IMA graft
which was experiencing vaso-steal phenomenon from a persistent anomalous lateral internal
thoracic artery. It was subsequently embolized with coils with improved perfusion
in LAD and symptomatic improvement.
A CASE OF MONDOR's DISEASE: SUPERFICIAL THROMBOPHLEBITIS OF THE BREAST
D. Cywinski
1; E. Caiola1. 1University of Rochester, Rochester, NY. (Tracking ID #117108)
LEARNING OBJECTIVES
1. Recognize that thrombophlebitis of superficial veins of the breast is an uncommon
condition that is usually self-limited. 2. Review the potential etiologies of Mondor's
disease: most commonly idiopathic, post breast surgery and uncommonly due to underlying
breast cancer. 3. Review that Mondor's disease can be diagnosed with color flow Doppler
examination of the breast and if no other abnormalities are detected can be followed
and treated symptomatically.
CASE
A previously healthy 26-year-old female presented with a two-day history of a painful
left breast with a palpable cord. She denied a history of breast-feeding, trauma,
fever or chills. She was G2P1011 with a 2-year-old child. Depo-Provera was her only
medication. She denied alcohol, tobacco or drug use. She had no previous history of
thrombophlebitis or deep venous thrombosis. Family history was negative for thromboembolism
or breast cancer. Physical examination was notable for an approximate 10-cm palpable,
tender venous cord over the upper outer quadrant of the left breast. There was minimal
surrounding erythema and induration. There were no palpable breast masses or axillary
adenopathy. There was no extension to the axillary veins and there was no arm edema
or asymmetry. The remainder of the exam was non-focal. A Doppler examination demonstrated
a hypoechoic tubular structure without vascular flow consistent with Mondor's thrombophlebitis.
No other abnormalities were detected. The patient was treated symptomatically with
warm compresses and NSAIDs and had complete resolution of her symptoms 4 weeks post
presentation.
DISCUSSION
Mondor's disease is a rare condition of superficial thrombophlebitis of the breast
veins. It is usually a self-limited condition that can be followed and treated supportively
with NSAIDs. In most cases a cause is not found but Doppler examination and possible
mammography are indicated. Mondor's disease may be a complication of breast surgery
and uncommonly associated with underlying breast cancer.
A CASE OF NON-MENSTRUAL STAPHYLOCOCAL TOXIC SHOCK SYNDROME
S. Arora
1. 1University of Connecticut, Farmington, CT. (Tracking ID #117432)
LEARNING OBJECTIVES
To recognize and manage toxic shock syndrome (TSS).
CASE
A 24-year old previously healthy male deli worker presented with a painful, marble
sized swelling posterior to right greater trochanter, high fever and vomiting for
2 days with generalized red skin rash involving the entire body for a day. He had
not passed urine for 12 hours. There was no preceding history of trauma or any outdoor
activity. Examination revealed tachycardia with HR of 140 bpm, fever with temperature
of 104oF and hypotension with BP of 80/58, pierced lower lip with lip ring, intensely
red, blanchable erythema involving the entire skin and oro-pharyngeal mucous membranes.
There was 2 × 2 cm tender, fluctuant swelling, mobile over underlying muscle located
5 cm posterior to right greater trochanter over the posterolateral aspect of right
hip. Incision and drainage of the swelling yielded 5 ml of yellow pus which grew staphylococcus
aurues sensitive to oxacillin. Investigations revealed WBC of 28,000/cmm with 18%
bands and 81% neutrophils, platelets: 90,000/cmm, BUN/Cr: 44/4.8 and FeNa of 0.4%,
the patient was diagnosed with staphylococcal toxic shock syndrome and was treated
with IV fluids, IV Vancomycin and supportive care of acute renal failure. The patient's
renal function started improving by D2, he became afebrile on D4 with gradual resolution
of rash subsequently. He was discharged on D4 on Dicloxacillin and recovered with
no sequelae.
DISCUSSION
Staphylococcal TSS is an acute life-threatening toxin-mediated intoxication caused
by TSS toxin 1 or staphylococcal enterotoxin B. Although menstruation remains the
most well-known setting for TSS, 50% of TSS is non-menstrual and can complicate the
use of barrier contraceptives, child birth, superinfection of various skin lesions
including burns, insect bites, varicella, surgical wounds and post-influenza pneumonia.
The primary site of colonization often appears entirely benign. CDC criteria for diagnosis
includes presence of all of the following: hypotension or orthostatic drop in BP,
temperature >102oF, diffuse macular erythroderma, desquamation of palms and soles
1–2 weeks after onset, negative results of blood, throat or CSF cultures which may
suggest an alternative diagnosis and involvement of at least three of the following
organ systems: gastrointestinal (nausea and vomiting), muscular (severe myalgias or
elevated CPKs, mucous membranes, renal, hepatic, hematological (thrombocytopenia <100,000,
central nervous system (disorientation but no focal neurological signs). Treatment
includes site drainage, aggressive fluid resuscitation, anti-staphylococcal antibiotics
for 14 days, pressors for hypotension and correction of dyselectrolytemia. Critically
ill or unstable patients benefit from intravenous immunoglobulin.
A CASE OF POLYMICROBIAL ENDOCARDITIS IN AN INTRAVENOUS DRUG ABUSER DUE TO ANAEROBES
S. Oh
1; N. Hussain1; P.R. Havlen1. 1University of Texas Medical Branch at Galveston, Galveston,
TX. (Tracking ID #115818)
LEARNING OBJECTIVES
1. Gain awareness of Infective Endocarditis (IE) due to anaerobic organisms 2. Compare
IE in intravenous drug abusers (IVDA) from other cases 3. Recognize that peculiar
habits of IVDA can result into unusual polymicrobial IE.
CASE
A 33-year-old white male presented to our hospital with a two-week history of subjective
fevers, chills, and rigors. He had history of intravenous drug abuse and a habit of
licking the needle to the dorsum of the tongue before injection into his arm. Blood
cultures grew Actinomyces odontolytica, Veillonella species, and Prevotella melaninogenica.
CT of the thorax showed multiple cavitary lesions in both lungs and echocardiogram
showed vegetations on the tricuspid valve. The patient was treated with a six-week
course of penicillin G and metronidazole. He responded well with complete resolution
of symptoms.
DISCUSSION
Endocarditis in intravenous drug users are usually right sided and of the tricuspid
valve. Right-sided endocarditis presents with a syndrome of persistent fever and pulmonary
symptoms due to septic emboli including cough, dyspnea, and hemoptysis. The peripheral
stigmata of endocarditis are not classically found in right-sided endocarditis. Although
the most common organism isolated is Staphlococcus aureus, it is important to consider
other more fastidious causes of infection in this population including those of endogenous
origin. Anaerobes are predominant components of normal human skin and mucous membranes
and are an uncommon cause of endocarditis. Most cases are caused by anaerobic cocci,
Propionibacterium acnes and Bacteroides fragilis group. Actinomyces odontolytica,
Veillonella species, and Prevotella melaninogenica reside predominantly in saliva
and the dorsum of the tongue as compared to other organisms. We believe that his peculiar
habit of licking the needle to the dorsum of the tongue to gauge the strength of the
injection, subjected our patient to infection by these particular anaerobes. Polymicrobial
endocarditis is a rare entity that is found almost exclusively in intravenous drug
abusers. Although uncommon, it is important to consider since it carries a mortality
rate exceeding 30%. There are documented cases in which cultures from the vegetations
grew more organisms than the blood cultures, further exemplifying the fastidious nature
of the organisms causing endocarditis in intravenous drug users. Therefore, some authors
recommend empiric coverage of both skin and oral flora when endocarditis is suspected
in this population. Penicillin G or other bactericidal agents appear to be the treatment
of choice for these three organisms. Metronidazole is often added due to the growing
resistance of anaerobes towards penicillins.
A CASE OF POST-OBSTRUCTIVE PNEUMONIA SECONDARY TO BRONCHOLITHIASIS
S.E. Luckhaupt
1; L. Coberly1. 1University of Cincinnati, Cincinnati, OH. (Tracking ID #115743)
LEARNING OBJECTIVES
1) Distinguish post-obstructive pneumonia from uncomplicated community acquired pneumonia
2) Recognize broncholithiasis as a cause of bronchial obstruction 3) Manage bronchial
obstruction to prevent recurrent pneumonia.
CASE
A 54-year-old male smoker with an unremarkable past medical history presented with
a 2-week history of shortness of breath, cough, purulent sputum, pleuritic chest pain,
and orthopnea. On exam, he had a temperature of 101.4, respirations of 28 and a pulse
ox of 89% on room air. Chest exam revealed bibasilar rhonchi and intermittent wheezing
over the left lung base. Initial laboratory data: WBC 20.5 with 12% bands, Hb 15.6.
ABG on room air: pH 7.44, pCO2 36, pO2 64. A chest x-ray suggested left lower lobe
consolidation with pleural effusion. Despite treatment with iv antibiotics, his oxygen
requirement increased and serial x-rays showed increasing infiltrate and effusion.
A CT on hospital day #3 revealed extensive loculated left pleural effusion with a
compressed lower lobe, possibly caused by calcified left hilar lymph nodes. An ultrasound
was negative for free-flowing fluid, so chest tubes were placed, and t-PA was used
to assist in drainage. A repeat CT showed improvement in the effusion, but compression
of the left lower lobe persisted. Bronchoscopy ultimately revealed obstructing broncholiths.
The broncholiths could not safely be removed, so left lower lobectomy was performed.
Pathology showed four hard tan-gray stones measuring 0.4 cm to 1.5 cm in diameter
and lymph nodes with necrotizing granulomas, negative for neoplasia. No fungi, acid
fast bacilli, or other organisms were identified in the pathology specimens or in
the pleural fluid.
DISCUSSION
This patient's presentation provided several clues that he did not have a typical
case of community acquired pneumonia. Despite having an unremarkable medical history,
he was very ill on presentation with hypoxemia, which progressed even after treatment
with antibiotics. Localized wheezing raised suspicion for bronchial obstruction and
concern about the possibility of carcinoma. Broncholithiasis is a less common cause
of bronchial obstruction, which usually presents with hemoptysis (from erosion of
pulmonary vessels), wheezing, shortness of breath, or chronic cough. It is often associated
with fungal infection, such as histoplasmosis, or tuberculosis. The cause of broncholithiasis
in this case was unclear. The diagnosis can usually be confirmed by bronchoscopy,
but bronchoscopic removal carries a high risk of bleeding, so surgical resection is
often required to relieve obstruction.
A CASE OF RAPIDLY FATAL ASPERGILLOSIS IN AN IMMUNOCOMPETANT PATIENT
F.K. Salahuddin
1; S. Chitavellue2; K. Karamchandanni3. 1University of Illinois at Peoria,SFMC., Peoria,
IL; 2University of Illinois College of Medicine,@Peoria,SFMC, Peoria, IL; 3University
of Illinois College Of Medicine,@Peoria, Peoria, IL. (Tracking ID #117272)
LEARNING OBJECTIVES
1. Diagnosis of massive hemoptysis. 2. Aspergilloma as a cause of hemoptysis. 3. Management
of life threatening hemoptysis using various means.
CASE
A 60 year old male was admitted into the hospital because of pleuritic chest pain,
hemoptysis, fever, lethargy and significant weight loss. He was a retired janitor
in a school. At the time of presentation, he was in respiratory distress and examination
revealed bilateral crackles and wheezes. Chest X-ray and CT scan showed consolidation
and cavitation of right upper lobe (Figure below). He underwent diagnostic flexible
bronchoscopy which confirmed the bleeding from right upper lobe without any intra-bronchial
pathology. Bronchoalveolar lavage grew aspergillious. Patient was treated with Amphotericin
B because of massive hemoptysis. He continued to have massive hemoptysis which required
mechanical ventilatory support with double lumen endotracheal intubation. Patient
was sent for an emergent bronchial arteriogram and had control of bleeding with coiling.
After 24 hours patient developed another episode of maasive hemoptysis which lead
to his demise. Autopsy confimed the angioinvasive aspergillosis.
DISCUSSION
Angioinvasive pulmonary aspergillosis is commonly seen as a serious complication in
immunosupressed individuals such as patients with AIDS and leukemia. It is rare to
encounter angioinvasive aspergillosis in immunocompetant individuals. Aspergillosis
can develop as a fungal ball in preexisting pulmonary cavities causing lifethreatening
massive hemoptysis. Routine surgical recection of aspergillious is not recommended
but should be reserved for patients with recurrent severe refractory hemoptysis. Pleuro-pneumonectomy
should be avoided. Lung necrosis can result from invasion of fungus into the vasculature,
leading to vascular thrombosis and hemorrhage. Massive hemoptysis can be managed with
mechanical ventilation using double lumen endotracheal tube, bronchial artery embolization
and or surgery. Prognosis in immunocompetant patients is usually good with above therapies.
A CASE OF RHODOCOCCUS EQUI PNEUMONIA IN A RENAL TRANSPLANT PATIENT
T.S. Bischof
1; J. Hariharan1; M. Graham1. 1Medical College of Wisconsin, Milwaukee, WI. (Tracking
ID #116015)
LEARNING OBJECTIVES
(1) To recognize the clinical presentation of atypical pneumonia in transplant patients.
(2) To educate the clinician on the presentation, radiography, pathology, and treatment
of Rhodococcus equi pneumonia.
CASE
A 48 y/o male with IgA nephropathy and 4 renal transplants presented with a one week
history of nausea, vomiting, and diarrhea. He related dehydration, weakness, low-grade
fevers, night sweats, and weight loss. He denied chest pain, shortness of breath or
cough. The patient was taking immunosuppressive and antihypertensive medicines. Physical
exam revealed an afebrile, normotensive, cachectic male in no acute distress. Exam
was within normal limits, and lungs were clear. BMP was normal except for Bun/Cr of
37 mg/dL and 2.0 mg/dL. WBC was 9.0, Hgb 11.9 g/dL, and urinalysis revealed no proteinuria
or white cells. Blood, urine and stool cultures were negative. Patient was hydrated,
and CXR revealed a new opacity in the left lung. A chest CT revealed a 4.5 × 2.3-cm
consolidation in the left lower lobe, but was negative for bony lesions and lymphadenopathy.
A bronchoscopy and CT guided biopsy were done, and cultures from both subsequently
grew Rhodococcus equi. On directed questioning, it was found the patient lives near
a farm with routine exposures to horses and had a new dog. Therapy with moxifloxacin
and azithromycin was planned until the lesion cleared on repeat CT scan.
DISCUSSION
Rhodococcus equi is a gram-positive coccobacillus that usually causes infections in
grazing animals. Infection in humans is rare, but over 100 cases have been reported.
Rhodococcus is often overlooked in cultures as a non-pathogenic organism and its insidious
onset often leads to delays in diagnosis. Pulmonary infection is the most common,
and symptoms include fever, cough, and weight loss. On radiography, the superior lobes
are mainly involved, and cavitation is frequent, as well as effusion and empyema.
Diagnosis is based on positive culture. Most isolates are susceptible to erythromycin,
ciprofloxacin, and aminoglycosides. Oral and parenteral combinations of the above
are used for treatment for at least two months. This patient was treated for 5 months
and repeat CT 3 months later showed decreased consolidation. It is well known that
immunocompromised patients are more prone to atypical infections. This case represents
a rare cause of a treatable bacterial infection in a transplant patient and the value
of social and personal history in medical management. It is important to recognize
that when patients present with vague complaints and lack of physical signs, a good
history and continually pursuing identification of treatable causes is important.
Rhodococcus equi pneumonia is rare but understanding the nature of its presentation
is highlighted in this case.
A CASE OF UNSTABLE ANGINA IN A YOUNG MAN
B. Barmar
1; G. Tabas1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #103913)
LEARNING OBJECTIVES
Learning Objectives: (1) Recognize angina in young patients. (2) Consider cardiac
and noncardiac causes of chest pain in young patients.
CASE
A 30-year-old Korean man with a history of gastritis and gastroesophageal reflux disease
presented to his primary care physician's office with exertional chest pain relieved
by rest. Because of his underlying gastrointestinal problems, young age, and lack
of cardiac risk factors, his physician prescribed pantoprazole, obtained a complete
blood count (CBC), and scheduled him for a treadmill stress test later that week.
The next day, when the CBC revealed a platelet count of 2,800,000/uL (normal range
150–450,000/uL) the patient was instructed to go to the emergency department. He reported
chest pain at rest, but this resolved after 2 sessions of emergent platelet pheresis
and treatment with aspirin and nitroglycerin. Cardiac enzyme levels and electrocardiographic
findings were normal. Peripheral smear showed numerous platelets, and bone marrow
biopsy confirmed the diagnosis of essential thrombocytosis. After the initiation of
anagrelide, a platelet-reducing agent, the patient's platelet count dropped to 1,700,000/uL
by the second hospital day. To avoid a positive stress test result attributable only
to platelet sludging in the coronary arteries, the physician waited until the platelet
count was below 600,000/uL to perform a stress test. When the test was performed,
it yielded negative results for ischemia. At 8 months after diagnosis, the patient
is asymptomatic and has a platelet count of about 300,000/uL.
DISCUSSION
In young patients, cardiac causes of chest pain can be found in about 16% of cases,
noncardiac causes in 68%, and unknown causes in 16%. The common noncardiac causes
include musculoskeletal problems (in 36% of cases), gastrointestinal problems (in
19%), psychological problems (in 8%), and pulmonary problems (in 5%). The noncardiac
causes that are most serious and require immediate treatment are pulmonary embolus,
pneumothorax, and aortic dissection. A thorough history and physical examination and
focused laboratory studies usually exclude life-threatening causes of cardiac chest
pain. Although the patient in this case was young, his chest pain was typical for
angina, so further investigation was initiated. Investigation uncovered essential
thrombocytosis, an unusual cause of angina.
A CASE OF WIDE ANION GAP NON-ACIDOSIS
E. Cichowski1; H. Sakowski
1; H. Hashish1; R. Baltaro1. 1Creighton University, Omaha, NE. (Tracking ID #117266)
LEARNING OBJECTIVES
1) Recognize laboratory error in the measurement of serum bicarbonate. 2) Utilize
the Henderson-Hasselbach equation to indentify blood gas analysis errors. 3) Identify
a previously unrecognized interfering substances as potential causes of laboratory
errors.
CASE
A 72 year-old male was admitted for respiratory distress and confusion, and found
to have a right upper lobe lung mass and hypercalcemia. He was intubated on the second
hospital day due to worsening of his respiratory status. Propofol was initiated for
sedation and methylprednisolone and levofloxacin were given for a presumed post-obstructive
pneumonia. His initial arterial blood gas after intubation showed a pH 7.38 pCO2 38
pO2 143 on an Fio2 of .60. His measured HCO3 was 26 meq/l. Over the next 4 days, his
measured bicarbonate progressively dropped to 8 meq/l despite no change in his arterial
blood gas (pH 7.38 pCO2 36 pO2 103 on an FiO2 of .45). His anion gap was calculated
at 19. Serum lactate was normal, and serum ketones were absent. Consultation with
the pathology department revealed the patient's serum to be grossly lipemic. A review
of the chart revealed the patient did receive lipid infusions with TPN 36 and 18 hours
prior to this discovery. A lipid panel was obtained and revealed marked hypertriglyceridemia
at 4,426 mg/dl. The lipid infusions were discontinued, and the propofol was weaned
off. The bicarbonate level dropped to a low of 3 meq/l approximately 7 hours after
the medication was discontinued. Four hours later, the bicarbonate had corrected to
21 meq/l. The serum, however, remained grossly lipemic. The patient's condition continued
to decline with the development of septic shock, multi-organ failure and ventricular
arrythmias. Results of a previous bronchoscopy demonstrated small cell carcinoma.
The patient's family requested no further aggressive treatment and he expired later
that day.
DISCUSSION
This patient developed marked derangement in his measured bicarbonate levels that
did not correspond to his arterial blood gas analysis (according to the Henderson-Hasselbach
equation). A laboratory error was hypothesized as the cause. Due to the finding of
lipemic serum, the hypertriglyceridemia was initially suspected as the interfering
substance. Upon discontinuing the propofol, the serum bicarbonate level normalized,
the serum, however remained lipemic. In a review of the literature, neither propofol
nor hypertriglyceridemia have been reported as potential causes of this lab error.
Further testing is needed to determine the role of propafol as an interfering substance
in bicarbonate laboratory analysis.
A CASE REPORT OF OXYGEN EMBOLISM FOLLOWING HYDROGEN PEROXIDE INGESTION
D. Misra
1; B. Legere1. 1New Hanover Regional Medical Center, Wilmington, NC. (Tracking ID
#116225)
LEARNING OBJECTIVES
Recognize that ingestion of concentrated solution of hydrogen peroxide can result
in significant morbidity and mortality owing to venous or arterial oxygen embolization.
We intend to share our experience through a case report to emphasize this fact .
CASE
We present the case of an 82 yr old caucasian female with prior history of emphysema
who had inadvertently ingested a large quantity of concentrated hydrogen peroxide
solution. Following this, she vomited and developed resiratory distress which required
intubation and mechanical ventilation. On examination, she was sedated, tachycardic
and had hemoccult positive stool. Blood work revealed elevated white cell count and
a low hematocrit. Her basic metabolic panel, urine drug screen, liver function tests
were within normal limits. Chest xray showed emphysema. CT scan of abdomen/pelvis
was significant for portal venous gas and pneumatosis involving duodenal and jejunal
wall. Upper endoscopy revealed hemorrhagic gastritis and distal esophagitis. She was
started on empiric antibiotics and followed with serial abdominal radiographs. CT
scan of the abdomen obtained five days later revealed no free air or pneumatosis.
On the sixth day of hospitalization, patient was extubated and at that point of time
was noted to have right sided hemiparesis. MRI scan of the brain revealed multiple
areas of acute/subacute non-hemorrhagic infarction. Patient was evaluated by a neurologist
and it was felt that her neurologic deficits were a result of oxygen embolization.
Patient gradually improved with physical and occupational therapy and currently awaits
discharge to a rehabilitation facility.
DISCUSSION
Hydrogen peroxide is widely used as an oxidant/disinfectant. It is sold in health
food stores also as means of “improving oxygenation” in people with coronary artery
disease. Literature search revealed several cases of accidental hydrogen peroxide
ingestion. A retrospective review of all exposures reported to a poison control center
revealed that 0.34% were hydrogen peroxide related. Although exposure to diluted (3%)
hydogen peroxide is benign, ingestion of the concentrated form can be dangerous. Following
ingestion, hydrogen peroxide breaks up into water and oxygen in the presence of catalase.
When the amount of oxygen produced exceeds the maximum blood solubility, embolization
occurs. We emphasize that physicians should be alert to the possibilty of multiorgan
embolization in patients presenting with accidental ingestion of concentated hydrogen
peroxide.
A CASE REPORT OF RECURRENT COCCIDIOIDES MENINGITIS (CM)
S.M. Maiorano
1; P. Radhakrishnan2. 1St. Joseph's Medical Center, Phoenix, Phoenix, AZ; 2Catholic
Healthcare West, Phoenix, AZ. (Tracking ID #117418)
LEARNING OBJECTIVES
1. Recognize that CM recurrence can occur despite prolonged antifungal treatment.
2. Recognize that indwelling CFS shunt can mask the hallmark symptoms of hydrocephalus
associated with CM. 3. Recognize that diagnosis of CM can be made on serum serologies
without positive CSF cultures.
CASE
A 62 year old male, presented with a 2 month history of worsening diplopia, ataxia
and headache. Past History-CM with obstructive hydrocephalus and VP shunt. He was
treated with Amphotericin B (intrathecal and systemic) for 2 years followed by Fluconazole
for 8 years. He had been off Fluconazole for the last 5 years. Physical exam—He was
somnolent, but arousable. Eyes-limited upward movement with downbeating nystagmus,
disconjugate gaze with mild right lateral ocular deviation. Lab. data-CT head—mild
right encephalomalcia, enlargement of 3rd and 4th ventricles, catheter in the right
lateral ventricle. CSF-(from the shunt and a lumber puncture)-including Gram stain-negative.
Positive CSF Coccidioides IgG and serum IGG,IGM antibodies. Complement fixation (CF)
titer 1:64. Catheter tip-Coagulase negative Staphylococcus. MRI of the head—ventriculomegaly,
increased periventricular and meningeal enhancement. He was diagnosed with recurrent
CM, shunt failure due to presumed Staphyloccal infection. He was started on Voriconazole
and Vancomycin. The shunt was replaced. He improved with resolution of his neurological
symptoms and signs. He was discharged with the plan to continue the Voriconazole indefinitely.
DISCUSSION
CM is a grave form of disseminated Coccidiodes infection. Of the nearly 100,000 cases
per year, only 0.1 percent present as meningitis. This case has several interesting
aspects, the first being the recurrence of the CM after several years. Recurrences
usually occur shortly after discontinuing therapy, as despite adequate antifungal
penetration the fungus is not easily cleared. In this case, the patient remained symptom
free for 5 years after stopping therapy. Second, the temporal association of shunt
blockage and recurrence of symptoms of CM made us postulate that the patient remained
symptom free due to the drainage of CSF and clearance of the fungus. Little data is
available as to the incidence or common etiologies of shunt failure, but many case
reports have found bacterial shunt obstruction through colonization as well as fungal
biofilm occlusion. Third, the diagnosis of CM recurrence was made based on the CSFand
serum studies. As CSF cultures are positive in only one third of cases, positive CSF
IgG or IgM and CF antibodies are very helpful in diagnosing CM in patients with a
high pre-test probability and negative cultures. While there are definitive guidelines
for the duration of treatment of CM, patients who experience a relapse should be continued
lifelong therapy.
A DIAGNOSIS AT BOTH ENDS: A CASE OF CELIAC DISEASE AND MICROSCOPIC COLITIS
D. Nataraj
1; R. Granieri1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115730)
LEARNING OBJECTIVES
(1) To recognize the clinical history and histopathology of celiac disease and microscopic
colitis (2) To recognize an association of celiac disease with microscopic colitis
(3) To manage celiac disease and microscopic colitis.
CASE
A 31 year-old female presents with 6 months of nausea, bilious emesis, abdominal cramps,
watery diarrhea, and 30 lb weight loss. Diarrhea occurs 5 to 15 times daily. Physical
examination reveals cachexia, tachycardia, dry mucous membranes, normal bowel sounds,
and abdominal distention with mild but diffuse tenderness to palpation. Initial laboratory
values demonstrate hypokalemia, contraction alkalosis, normal amylase and lipase,
hypoalbuminemia, normal liver function tests, hemoglobin of 13, leukocytosis of 22,000,
and urinalysis suggestive of an infection. Additional studies show low iron level,
increased RDW and decreased % saturation. EGD and colonoscopy are grossly normal.
Colonic biopsy reveals increased intraepithelial lymphocytes. Duodenal and jejunal
biopsies show villous atrophy with cryptitis, and mucosal lymphocytes and plasma cells.
She is found to have antigliadin and antiendomysial antibodies. The diagnoses of both
celiac sprue and microscopic colitis are made. With initiation of a gluten/lactose-free
diet, prednisone, and octreotide, her symptoms improve considerably.
DISCUSSION
Iron deficiency anemia is the most common presentation of celiac disease. Abdominal
discomfort and bloating, also common features, often incorrectly lead to the diagnosis
of irritable bowel syndrome. Significant diarrhea is present in 50% of patients. Serologic
studies including antiendomysial antibody (sensitivity 85%–98%; specificity 97%–100%)
and tissue transglutaminase antibody (sensitivity 90%–98%; specificity 95%–97%) have
facilitated diagnosis of this disease; however, the gold standard remains small bowel
biopsy. Standard therapy is dietary gluten restriction, which results in symptomatic
improvement in 70% of patients within 2 weeks and a decrease in antibody titers within
6 months. Conditions associated with celiac disease include type 1 diabetes and microscopic
colitis (either lymphocytic or collagenous). Lymphocytic colitis typically presents
in the sixth decade as watery diarrhea. The diagnosis is made by colonic biopsy revealing
increased intraepithelial lymphocytes. Medications such as lansoprazole and NSAIDS
have been associated with this condition. Large randomized controlled trials regarding
treatment have not been conducted. Therapy is based on case reports involving small
numbers of patients and includes removal of the offending drug and addition of antidiarrheals,
aminosalicylates, octreotide, prednisone, or budesonide. A prospective study of 81
patients treated with a variety of the above medications demonstrated a 70% resolution
of diarrhea. Bismuth subsalicylate has been promising in small trials.
A DIFFERENT TWIST TO AN ABDOMINAL PAIN
C.E. Landaverde
1; S. Dea1. 1UCLA-San Fernando Valley Program, Sylmar, CA. (Tracking ID #115130)
LEARNING OBJECTIVES
1) Recognize that a volvulus can have an atypical age of presentation and occur in
someone with no predisposing risk factors. 2) Recognize clinical and radiological
features of a volvulus.
CASE
A 39 y/o Hispanic nulliparous female presented to the emergency room with a one day
history of abdominal pain, distension, nausea and vomiting. The abdominal pain was
described as sudden onset, continuous, severe, crampy, lower abdominal pain worse
with eating. The patient reported having explosive, watery, brown diarrhea soon after
the onset of the abdominal pain for a couple of hours but since had not had a bowel
movement nor passed flatus. Patient denied prior history of constipation or use of
psychotropic medications or a diet high in fiber. The patient was afebrile with a
blood pressure of 168/56. The abdominal exam revealed moderate distension, no bowel
sounds, tympanic with tenderness to palpation in the lower abdominal quadrants, left
more than the right. There was no rebound tenderness or guarding. The rest of the
physical exam was unremarkable. Laboratory results were unremarkable except for a
bicarbonate level of 31. An abdominal plain film revealed a dilated, ahaustral loop
of large bowel extending from the pelvis to the right upper quadrant in an “inverted
U” appearance. A CT scan of the abdomen confirmed the diagnosis of a sigmoid volvulus
with findings of a dilated sigmoid colon. A gastrograffin enema revealed a partial
sigmoid volvulus, which had reduced upon the post-evacuation examination. Subsequently,
the patient had a sigmoid resection with primary anatamosis performed.
DISCUSSION
Sigmoid volvulus is produced when a long redundant sigmoid twists about its mesenteric
axis in either direction and forms a partial or complete loop obstruction. It occurs
more commonly in the elderly, individuals with neurologic conditions, and in patients
in nursing homes or mental health facilities. The common factor is chronic constipation.
Other predisposing risk factors include megacolon, an excessively mobile colon, high-roughage
diet, and lead poisoning. Furthermore, volvulus has been observed to occur most commonly
in young patients in settings such as Crohn's disease, pregnancy, Chagas and in individuals
with prior history of roundworm infestation. Patients present with abdominal pain,
distension, nausea and absolute constipation with vomiting as a late sign. Findings
on abdominal plain films include a markedly distended sigmoid loop, inverted U-shaped
appearance, loss of colonic haustra and elevation of the sigmoid loop under one of
the diaphragms. The involved bowel walls are edematous, resulting in a coffee bean–shaped
structure (the “coffee bean” sign). CT findings of ischemia in a sigmoid volvulus
include the “whirl sign”, which represents tension on the tightly twisted mesocolon
by the afferent and efferent limbs of the dilated colon.
A FATAL CASE OF VARICELLA-ZOSTER PANENCEPHALO-MENINGO-RADICULO-MYELITIS IN A PATIENT
WITH AIDS.
D.B. Van Schyndel
1. 1Hennepin County Medical Center Internal Medicine Dept., Minneapolis, MN. (Tracking
ID #117292)
LEARNING OBJECTIVES
1. Recognize that varicella-zoster virus infection of the central nervous system is
a sign of probable immunocompromise. 2. Recognize that the characteristic rash often
seen in varicella-zoster infections may not appear in immunocompromised patients.
3. Diagnose varicella-zoster infection of the central nervous system using PCR amplification.
CASE
A 38 year old previously healthy Canadian woman presented to the emergency department
with a four-day history of frontal headache and one day of lower extremity weakness
and numbness. The initial exam revealed 4/5 lower extremity strength. The initial
head CT was normal and a lumbar puncture was performed. CSF studies revealed increased
protein and white blood cell counts. Empiric acyclovir was started. Six hours after
the patient was admitted, she complained of worsening leg weakness and numbness extending
to her chest. On exam she was areflexic in her lower extremities, paraplegic, and
had a sensory level at T4. She became hypoxic and was intubated. Several hours later
the patient was reexamined and no brain stem reflexes were present. A repeat head
CT revealed brain stem swelling and leptomeningeal enhancement of the brain stem and
cerebellum. The next day, the varicella-zoster PCR performed on cerebrospinal fluid
was found to be positive. The patient's family reported that she had been exposed
to a child with chicken pox two weeks before her admission. They did not remember
the patient complaining of a rash. An HIV test was also positive. A MRI showed changes
consistent with acute disseminated encephalomyelitis. Her neurologic exam was unchanged.
The patient's family decided to withdraw support and the patient died approximately
72 hours after admission. An autopsy revealed lymphocytic encephalomeningoradiculomyelitis.
DISCUSSION
Zoster is not viewed as an AIDS-defining illness, but it can indicate immunodeficiency
and tends to occur more often in patients with HIV. Varicella-zoster virus is likely
to be associated with HIV in central and east Africa, where the positive predictive
value of a history of VZV can be up to 90%. Among opportunistic CNS infections in
AIDS patients, VZV accounts for 2–4% of neurological disease. In one series of 11
AIDS patients with VZV encephalitis, four did not report a rash. Health care providers
should therefore keep VZV on their differential in patients with HIV risk factors
who present with neurologic symptoms but do not report a rash. Examination of the
CSF usually reveals mild mononuclear pleocytosis, a normal or elevated level of protein,
and a normal glucose level. Varicella-zoster virus cannot be cultured from cerebrospinal
fluid, but the virus can be detected with PCR. The varicella-zoster PCR has a specificity
of 98.6% and a sensitivity of 100%.
A FIRM HANDSHAKE. PRESENTATION OF AN ECTOPIC GROWTH HORMONE SECRETING TUMOR
M. Chan
1; M. Ziebert1. 1Medical College of Wisconsin, Milwaukee, WI. (Tracking ID #116152)
LEARNING OBJECTIVES
1. Recognize the importance and early diagnosis and treatment of acromegaly. 2. Recognize
the importance of a good history and physical exam. 3. To develop a basic understanding
of pathophysiology and current treatment modalities.
CASE
A 58-year old Caucasian woman who presented to establish primary care was noted to
have very large hands on initial introduction. Her only complaints were chronic bilateral
hip and knee pain. Past medical history included hypertension, hypercholesterolemia,
and scoliosis. Review of systems revealed difficulty sleeping with excessive daytime
sleepiness, back pain, headaches, and polyuria. Physical exam revealed a woman with
significant mandibular overgrowth and prognathism, a deep resonant voice, a large
fleshy nose and very large hands. The patient was asked to bring an old ring and pictures
for comparison. Laboratory studies included a basic metabolic panel, complete blood
count, TSH, prolactin, FSH, LH, cortisol, ACTH, growth hormone (GH), and somatomedin
C or insulin-like growth factor-1 (IGF-1). Both GH and IGF-1 were extremely elevated,
up to five times the upper limit of normal. An MRI of her pituitary revealed a large
1.7 cm ectopic tumor in the sphenoid sinus. The patient was referred to endocrinology,
neuroophthalmology, and neurosurgery for evaluation. The patient subsequently underwent
sublabial, transphenoidal resection of her tumor and pathology confirmed isolated
GH producing cells. The patient currently feels like a “new person”. Her arthralgias,
headaches, and probable obstructive sleep apnea have significantly diminished. She
is currently on cabergoline or Dostinex and finishing her adjuvant radiation.
DISCUSSION
Acromegaly is a rare, chronic syndrome that is often diagnosed by the general internist.
Most commonly, it is caused by excessive secretion of GH by the somatotroph adenoma
of the anterior pituitary. However, very rarely, ectopic tumors may secrete GH and
present in an indolent fashion. Diagnosis is usually delayed for many years resulting
in significant morbidity and mortality. In the era of healthcare reform, the emphasis
is on a problem focused clinical encounter. This case illustrates that a rare, debilitating
disease can be diagnosed by simply shifting the focus back to the patient. A firm
handshake or first impression can still be a valuable clinical tool.
A GIFT FROM THE TOOTH FAIRY
D. Blackmon
1; M. Panda1. 1University of Tennessee, Chattanooga, chattanooga, TN. (Tracking ID
#106696)
LEARNING OBJECTIVES
To recognize the similarities in clinical and radiographical presentation of pulmonary
actinomycosis and neoplasms.
CASE
53 year old male with a heavy tobacco history, presented with malaise, non-productive
cough and weight loss for 3 months. On exam he had normal vitals, appeared non-toxic
but cachetic, with dental caries and diminished breath sounds on the left. Labs were
only significant for an elevated wbc count with microcytic anemia. CXR showed opacity
in left hemithorax. CT chest revealed 10 × 10 × 7 cm necrotic mass abutting the pericardium
and pleural suspicious for carcinoma. Biopsy revealed no neoplastic cells. Aspirate
cytology revealed filamentous sulfur granules consistent with Actinomycosis confirmed
by culture. Patient was treated sucessfully with penicillin and dental extractions.
DISCUSSION
Actinomycosis is a gram-positive anaerobic filamentous bacteria. Humans are the only
host. It resides in the oropharynx, GI and female genital tract and commonly causes
cervicofacial infections. Aspiration of oropharyngeal secretions commonly cause pulmonary
actinomycosis—50% of which is associated with dental caries. These bacteria invade
bony structures and cross-anatomic borders, making its appearance similar to neoplasms.
Diagnosis is by identification of “sulfur granules” on cytology or isolation of organism
on culture. Bronchoscopy, CT guided biopsy/aspiration or thoracotomy is often required
for diagnosis due similarity in presentation to neoplasm. Treatment requires PCN for
12 months and extraction of dental caries when indicated. Diagnosis of Actinomycosis
requires a high clinical index of suspicion and must be considered in individuals
with lung masses and poor dental hygiene in order to spare the patient from unnecessary
tests and invasive procedures.
A HIGHLY FUNCTIONING CASE OF DEMENTIA
G. Prakash
1; P. Koneru1; R.D. Hobbs1. 1Oakwood Healthcare System, Dearborn, MI. (Tracking ID
#117199)
LEARNING OBJECTIVES
To recognize a common error in making the diagnosis of dementia.
CASE
A 74-year-old woman with Alzheimer's disease presented for a physical exam. She had
been institutionalized in another city and had recently moved to be near her sister.
Her history was significant for resection of a pituitary tumor with resultant hypopituatrism,
hypogonadism, hypothyroidism and later, diabetes mellitus. She was a nurse by profession.
Her husband had died two years before. Physical examination revealed a dysconjugate
gaze, a dilated fixed right pupil and a visual field defect. During the exam she remarked
“Oh, you're checking my visual fields by direct confrontation.” She then explained
how these findings were “chronic since 1955.” She was alert and oriented, performed
serial sevens accurately, interpreted proverbs abstractly, and had only minor difficulty
remembering a name and address. When asked to spell “world” backwards she did so and
then asked the examiner if he would like to hear the alphabet spelled backwards. Without
an error or pause, she then accurately spelled the alphabet backwards. The examiner
later remarked jokingly, “This was the most highly functioning case of Alzheimer's
disease” that he had ever seen. Her miraculous improvement had occurred after moving
closer to her sister.
DISCUSSION
Studies done during the 1970's showed that between 10–20% of nursing home patients
diagnosed with dementia were actually suffering from untreated depression. Unfortunately,
since most dementia is incurable, such a diagnosis frequently labels an individual
as medically untreatable and condemns them to their continued existence with scant
hope of improvement. With more modern care the contribution of depression to dementia
has been recognized and is now frequently treated. Our patient did not have Alzheimer's
disease but was suffering from severe bereavement and isolation that improved when
she moved nearer her sister. This case should serve as a cautionary tale to clinicians
and underscore the point that in 2004 there are still individuals whose severe depression
can mimic dementia to the point of institutionalization.
A HIP FRACTURE ALREADY?
C. Christopher
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117403)
LEARNING OBJECTIVES
1. Recognize risk factors for osteoporosis in a young woman 2. Distinguish causes
of secondary osteoporosis.
CASE
A 44 year-old woman was admitted following a displaced left femur fracture. She also
noted four months of irregular menses and depression with poor appetite. Her body
mass index was 21, and the early fracture prompted an evaluation for osteopenia. She
smoked but did not consume alcohol. She noted a past history of a stomach ulcer that
required surgical intervention. She had no pallor, thyromegaly, or dental caries.
Her breast exam was normal. Her calcium level was 7.6 mg/dL, albumin 1.7 g/dL, phosphorous
3.0 mg/dL, alkaline phosphatase 279; her renal function and CBC were normal. An intact
PTH level was 53 pg/mL (normal 8–97). Her TSH was 1.65 uIU/mL with a free T4 of 0.50
ng/dL (normal 0.8–1.9). FSH, LH, and estradiol levels were consistent with premenopause.
Her 1,25-dihydroxycalciferol was 12.5 (15.9–55.6); the 25-hydroxycalciferol was 8.4
(8.9–46.7). Bone densitometry showed T-scores of −2.8 (hip) and −3.3 (spine). Alendronate
therapy was initiated with supplemental calcium and vitamin D. An extensive past medical
history revealed that the surgery for the duodenal ulcer required a Bilroth I anastomosis,
later revised to a Roux-en-Y re-anastomosis.
DISCUSSION
Risk factors for osteoporosis include gender, race, tobacco use, alcohol consumption,
low body weight, and nulliparity. Our patient's young age for a hip fracture prompted
an evaluation of secondary etiologies of osteoporosis. These include renal or liver
disease, malignancy, primary hyperparathyroidism, vitamin D deficiency, malabsorption,
malnutrition, myeloma, and hyperthyroidism. In our patient, the Roux-en-Y anastamosis
had led to malabsorbtion of fat-soluable vitamin D. She was started on parenteral
vitamin D in addition to alendronate and calcium supplements. Diagnosis of osteoporosis
is based on T-scores from bone densitometry studies that compare the patient to sex
and race matched young controls. T-scores below −1.5 is the recommended level for
therapy in patients with risk factors, and therapy should begin at T-scores below
−2.0 in the absence of risk factors.
A LARGE SPLENIC CYST: “INCIDENTALOCYST”
A. Sequeira
1; N.K. Atray1; T.J. Vachharajani1. 1Louisiana State University Medical Center at
Shreveport, Shreveport, LA. (Tracking ID #116793)
LEARNING OBJECTIVES
To discuss the differential diagnosis of an incidental splenic cyst.
CASE
A 31-year old type 1 diabetic male presented with a 5 day history of nausea, vomiting,
diarrhea and upper abdominal pain, 2 months after a motor vehicle collision. He denied
any prior symptoms of gastroparesis. Examination was remarkable for an afebrile patient
with epigastric tenderness. Laboratory data: Hb 13.3 g/dL, WBC 15 k/cmm, serum amylase
75 U/L, serum lipase 227 U/L, T.bil 0.5 mg/dL, albumin 3.9 mg/dL, Alk phos 126 U/L,
SGOT 12 U/L, SGPT 56 U/L, BUN 28 mg/dL, Creat 1.9 mg/dL, anion gap 18, urine ketones
4 + and an ABG with pH 7.31, PaCO2 27, PaO2 120, HCO3 12, SaO2 99% on 1.5 liters oxygen.
His symptoms of nausea, vomiting and abdominal pain persisted despite correcting his
ketoacidosis. A CT abdomen showed a calcified multiseptate splenic cyst measuring
12 × 8 cm, which was compressing the stomach. The possibility of a splenic abscess
precipitating ketoacidosis was entertained. His blood cultures were negative for bacteria,
fungi and acid-fast bacilli. The splenic aspirate was sterile for any organisms. Subsequently,
he underwent splenectomy for multiseptated cystic spleen with pressure symptoms. Pathology
revealed a 490 gm spleen measuring 16 × 13 × 8 cm. Histopathology revealed a cyst
without lining cells with organized fibrin and old hemorrhages, suggestive of a posttraumatic
pseudocyst.
DISCUSSION
Splenic cysts are rare, many of which are asymptomatic and incidental findings. They
are classified as true or false based on the presence or absence of an epithelial
lining. In the absence of an Echinococcal infection, cysts are commonly congenital
or post traumatic. The above case highlights the need to suspect posttraumatic splenic
cyst as a possible differential in a patient with a LUQ mass following an abdominal
trauma. As in the above case, large splenic cysts may mimic the symptoms of gastroparesis
in a diabetic.
“A LAZY HOUSEWIFE”: CASE OF LUPUS PNEUMONITIS
J.E. Cho
1; D. Yick1. 1University of California, Los Angeles—San Fernando Valley Program, Sylmar,
CA. (Tracking ID #115612)
LEARNING OBJECTIVES
1) Recognize lupus pneumonitis as an etiology of pulmonary effusion 2) Describe the
typical presentation of lupus pneumonitis 3) Recognize the treatment options and prognosis
of lupus pneumonitis.
CASE
A previously healthy 22 year old female presented to the emergency department with
acute shortness of breath. Her shortness of breath was worse with exertion and associated
with pleuritic chest pain for two days. She also noted fever, non-productive cough,
nausea, vomiting, and arthralgia. On presentation, she was febrile with temperature
38.6, blood pressure 88/54, pulse of 104 beats per minute, respiratory rate of 28
and oxygen saturation of 88% on room air improved to 92% with 2 liters of supplemental
oxygen. She was in moderate respiratory distress; however, she was speaking in full
sentences. Physical examination revealed absent breath sounds throughout right thorax
with decreased breath sounds half way up on the left thorax. There was associated
egophony, decreased fremitus, and dullness to percussion on the right thorax. There
was no jugular venous distension, lower extremity edema, or skin rash. Chest radiograph
revealed small pleural effusion on the left side one-fourth way up in addition to
the right-sided pleural effusion three-fourths way up with mediastinal shift to the
left. She was admitted to the intensive care unit for acute respiratory distress.
Chest CT with contrast confirmed findings of right-sided pleural effusion and small
left sided effusion. Thoracentesis was performed and it showed negative culture, gram
stain, and cytology with increased LDH and protein consistent with Light's criteria
for exudative process. The pleural fluid was positive for ANA at 1:10,000, lupus anticoagulant,
anti double stranded DNA 1:40, and anticardiolipin antibody. The diagnosis of lupus
pneumonitis was made, and she improved on intravenous solumedrol, and discharged home
on oral prednisone several days later.
DISCUSSION
Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disorder that may
affect one or multiple organ systems. Acute lupus pneumonitis is an uncommon presentation
that appears in 1% to 10% of the cases, and the symptoms include severe dyspnea, tachypnea,
fever, pleurisy, cough, basilar rales, hypoxia, and no apparent infection with radiographic
findings of bilateral lower lobe infiltrate and atelectasis. In addition, pleural
effusion is observed in up to 30% of cases. Lupus pneumonitis responds with glucocorticoids.
However, intravenous pulse steroid therapy or immunosuppressive drugs may be considered
if no improvement in 3 days. Lupus pneumonitis may progress to pulmonary fibrosis
and eventually develop into pulmonary hypertension. The prognosis of lupus pneumonitis
is poor with short-term mortality approaching as high as 50% with persistent pulmonary
function abnormalities, including severe restrictive pulmonary defect.
A METABOLIC MESS: A CASE OF ETHYLENE GLYCOL POISONING
S. Khan
1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116218)
LEARNING OBJECTIVES
1. Recognize causes of potentially fatal alcohol intoxication. 2. Recognize clinical
features of ethylene glycol poisoning. 3. Review management of ethylene glycol posioning.
CASE
A 55 year old African American female with depression, hypothyroidism, and breast
cancer presented with altered mental status. She lives with her mother, but history
taking was limited by the mother's Alzheimer's disease. The mother reported that the
patient had been vomiting earlier in the day. In the emergency room, patient became
unresponsive and was intubated. Her vitals were T 96.9F, HR 96, BP 125/90. Her exam
revealed left, fixed pinpoint pupil, flaccid extremities, absent reflexes. Her labs
revealed: Na 161, K 3.6, Cl 120, HCO3 7, BUN 14, Cr 1.7, glucose 114, anion gap 34.
Her calculated osmolarity was 320, measured osmolarity 551 and osmolar gap 231. Her
lactate was 9.5 and ammonia 98. Her WBC was 15.7 (no shift), Hgb 14.4, platelets 151.
Her liver function tests and TSH were normal. Her urine showd calcium oxalate crystals.
ABG prior to intubation revealed pH 6.93 pCO2 21 HCO3 4. Head CT and LP were negative.
Her ethanol level was 31 mg/dl and ethylene glycol level was 900 mg/dl. She received
two doses of fomepizole and D5W with 3 amps of bicarbonate. She was then placed on
an ethanol drip. She subsequently improved, was extubated and transferred to inpatient
psychiatry after she admitted to drinking antifreeze.
DISCUSSION
Three alcohols can produce fatal intoxication: methanol, isopropanolol, and ethylene
glycol. All can increase the osmolal gap, but only methanol and ethylene glycol cause
an anion gap metabolic acidosis. Ethylene gylcol is a component of antifreeze and
solvents. The lethal dose is 100ml. Clinical presentation ranges from from drunkenness
to coma. Complications involve the heart, lungs and kidneys. Two types of urinary
calcium oxalate crystals can be seen: needle shaped and envelope shaped. The absence
of crystalluria does not preclude the diagnosis. Urine examination by Wood's light
may reveal fluorescence if the patient has ingested antifreeze which commonly contains
fluorescin dye. Ethylene glycol is metabolized to toxic metabolites: glycolic acid
and oxalic acid. Glycolic acid falsely elevates lactate. Management consists of supportive
care, prevention of drug absorption, bicarbonate, and antidotes. Fomepizole, which
rapidly inhibits alcohol dehydrogenase, is the drug of choice for ethylene glycol
and methanol intoxication. Ethanol can also be used, but is not as potent. Both treatments
need to be initiated quickly, prior to alcohol metabolism. In severe cases, hemodialysis
may be required.
A MIDDLE AGE WOMAN WITH WORSENING SHORTNESS OF BREATH
N. Latif
1; G.H. Tabas1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115826)
LEARNING OBJECTIVES
Recognize a non-embolic cause of pulmonary occlusive disease(PVOD) in hypoxemic patients.
CASE
A 55 year old white female with a history of coronary artery disease, insulin dependent
diabetes mellitus and hypertension presented with a one week history of worsening
shortness of breath, dry cough, wheezing and chest discomfort. She was treated with
azithromycin as an outpatient but because her symptoms did not improve she presented
herself to the emergency department. There, her temperature was 39 degrees C and her
physical examination revealed some neck stiffness. Lumbar puncture was performed and
cerebrospinal fluid examination was unremarkable. Her chest X-ray was interpreted
as normal. The patient was empirically treated with intravenous levofloxacin. In the
hospital her oxygen saturation decreased to 86% and a repeat chest X-ray showed bilateral
pleural effusions and pulmonary edema. Computerized tomography of the chest revealed
no pulmonary embolism. Because of the onset of atrial flutter, echocardiography was
performed and showed an increased pulmonary artery pressure of 55 mmHg. To diagnose
the cause of her pleural effusions and hypoxemia she underwent video assisted thoracoscopy
and lung biopsy that revealed pulmonary veno-occlusive disease (PVOD). She was treated
with prednisone and coumedin with symptomatic improvement.
DISCUSSION
PVOD is rare but important cause of hypoxemia and pulmonary hypertension. PVOD has
no known etiology, can present at any age group with equal male and female distribution.
The pathologic hallmark of PVOD is occlusion of small pulmonary veins by fibrous tissue;
large veins are rarely affected. Pulmonary arteries may exhibit moderate to severe
medial hypertrophy and alveolar capillaries may become engorged and tortous. Interstitial
fibrosis may develop in the pulmonary parenchyma. There is no curative therapy and
immunosuppressive agents including prednisone are of unproved benefit. Anticoagulation
may improve survival but long-term prognosis is poor.
A MULTIPLE SCLEROSIS-LIKE ILLNESS ASSOCIATED WITH LEBER's HEREDITARY OPTIC NEUROPATHY
A.J. Huang
1. 1University of California, San Francisco, San Francisco, CA. (Tracking ID #116277)
LEARNING OBJECTIVES
1. Review the clinical features of Leber's hereditary optic neuropathy (LHON). 2.
Recognize LHON as a risk factor for developing multiple sclerosis. 3. Distinguish
between LHON and the optic neuritis of multiple sclerosis.
CASE
A 27 year-old man with a family history of Leber's hereditary optic neuropathy (LHON)
presented for physical examination before enrolling in a rehabilitation program for
the visually impaired. The patient reported 2 years of progressive, bilateral central
vision loss, similar to a female cousin who had been diagnosed with LHON after genetic
testing confirmed the presence of a characteristic mitochondrial DNA mutation, G11778A.
Unlike his cousin, the patient also suffered from patchy numbness and clumsiness in
both hands and lower extremities, which he attributed to drinking too much alcohol
(over 8 pints of beer per day). Physical exam revealed severe bilateral optic atrophy
with an otherwise normal cranial nerve exam, decreased vibration and joint position
sense in both feet, and a slow and wide-based gait. Routine laboratory studies, including
TSH, B12, RPR, and HIV, were normal. The patient was counseled to stop drinking, but
continued to have problems with coordination and ambulation after discontinuing alcohol,
and returned to clinic 3 months later after a mechanical fall. Follow-up exam revealed
interval development of moderate spasticity in both lower extremities, a mildly positive
Romberg sign, and worsened vibration and joint position sensory defects. Electromyography
was negative for lower motor neuron abnormalities. An MRI showed multiple focal areas
of T2 prolongation in the periventricular white matter of the corpus collosum, brainstem,
and cervical spinal cord, consistent with a demyelinating disease such as multiple
sclerosis. The patient was referred to neurology for management of multiple sclerosis
associated with LHON.
DISCUSSION
Leber's hereditary optic neuropathy (LHON) is a mitochondrially transmitted disease
affecting young adults, with a male to female ratio of approximately 4 to 1. It is
characterized by subacute, bilateral, central vision loss resulting in permanent optic
atrophy, with relative sparing of peripheral vision. While a tentative diagnosis of
LHON can often be made based solely on patients' clinical history, fluorescein angiography
and electrophysiology studies may be helpful in confirming the diagnosis. Over 95%
of patients with LHON have one of three mitochondrial DNA point mutations, G3460A,
G11778A, or T14484C, but only 50% of men and 10% of women who harbor one of these
mutations develop the optic neuropathy. A multiple sclerosis (MS)-like illness has
been described in patients with LHON, especially those with mutation G11778A, in which
MRI and CSF findings are identical to those of the MS population in general. The vision
loss associated with LHON differs from the optic neuritis more commonly seen in multiple
sclerosis in that it is bilateral rather than unilateral, is not accompanied by eye
pain, is not associated with pupillary reflex defects, and rarely responds to corticosteroids.
Screening LHON patients for MS, particularly if they have neurologic symptoms other
than visual loss, may be appropriate if one accepts that immunomodulatory treatment
should be started early in MS.
A NECROTIC PENIS
M. Glass
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117485)
LEARNING OBJECTIVES
1. Recognize the clinical presentation of calciphylaxis. 2. Recognize the risk factors
for calciphylaxis.
CASE
A 52-year-old man with was admitted for necrosis at the tip of his penis. He had a
history of diabetic renal failure, and was scheduled for dialysis following a permacath
placement. He was afebrile and a systolic murmur was noted at the base and apex of
the heart. His labs were notable for a phosphate of 8.1, and a calcium of 8.3. An
echocardiogram revealed calcified mitral and aortic valves. The necrotic area was
treated with surgical debridement. The surgical pathology report described acute and
chronic inflammation with extensive coagulative necrosis consistent with calciphylaxis-induced
ischemia.
DISCUSSION
Calciphylaxis is the deposition of calcium-phosphate crystals in the setting of either
hypercalcemia or hyperphosphatemia. Deposition in peripheral arteries can result in
ischemia with subsequent peripheral necrosis. A calcium-phosphate product of greater
than fifty should prompt suspicion of this complication. The diagnosis is suggested
by ischemic skin lesions and is confirmed by biopsy showing arterial occlusion and
calcification without vascultic changes. In this case, a diagnosis of calciphylaxis
was suggested by the clinical presentation and his history of renal failure; the elevated
calcium-phosphate product of 67 sufficiently increased the pre-test probability to
prompt a skin biopsy. In the setting of a calcium-phophate product greater than 50,
physicians should consider calciphalaxis as a potential cause of vascular insufficiency
and valvular calcification.
A NOT-SO-BENIGN CASE OF PROSTATIC HYPERPLASIA
A.N. Githaiga
1; P.K. Han1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115075)
LEARNING OBJECTIVES
1) To recognize bladder diverticuli and spontaneous bladder perforation as possible
complications of benign prostatic hyperplasia (BPH). 2) To identify clinical findings
suggestive of bladder perforation.
CASE
A 69-year-old man with a history of mild BPH, colonic diverticulosis and hyperlipidemia
presented to his doctor's office with a two-day history of lower abdominal pain, dysuria,
urinary urgency and frequency. He was treated empirically with ciprofloxacin and tamsulosin,
but his symptoms progressed to include generalized abdominal pain and distension,
constipation, nausea and vomiting. He presented to the emergency room two days later,
in distress from pain. Vital signs were normal.On physical examination his abdomen
was distended and tympanitic with absent bowel sounds. There was severe generalized
tenderness but no peritoneal signs and no masses. Rectal examination revealed an empty
rectal vault and a firm, moderately enlarged and non-tender prostate gland. Four attempts
were made at bladder catheterization but the catheter failed to pass through the urethra.
Bladder ultrasound showed an empty bladder. Laboratory tests included WBC 12.2 K,
BUN 38, creatinine 5.2 (baseline 0.9), and normal serum electrolytes. Urinalysis showed
0–3 WBC, 5–10 RBC, and few bacteria. Plain abdominal X-ray demonstrated a dilated
transverse colon with absence of gas in the distal colon. A non-contrast CT scan showed
inflammatory changes around the mesentery and bladder; gastrograffin enema and renal
ultrasound were normal. CT scan of the abdomen and pelvis was repeated with IV contrast,
and demonstrated communication in the superior aspect of the bladder with a contrast-filled
collection in the extraperitoneal space, consistent with a bladder leak. A cystogram
was obtained, which demonstrated bladder perforation along with trabeculation and
diverticuli, consistent with chronic bladder outlet obstruction. These findings were
confirmed by cystoscopy. The patient was managed conservatively with bladder catheterization
and had rapid resolution of his symptoms and renal insufficiency.
DISCUSSION
BPH is a common condition, although serious complications are unusual. Chronic bladder
outlet obstruction, however, may occasionally cause urinary retention, bladder diverticuli,
and, rarely, spontaneous bladder perforation, which presents with progressive abdominal
pain. Intraperitoneal rupture may cause peritonitis; in this particular patient, however,
the perforation was extraperitoneal, and thus there were no signs of peritoneal inflammation.
The case illustrates that although bladder perforation presents dramatically, affected
patients may have minimal prior symptoms of bladder outlet obstruction; therefore,
clinicians should be aware of this potential complication.
A PAIN IN THE BUTT
H. Segrest
1; J. Brice1. 1Tulane Health Sciences Center, New Orleans, LA. (Tracking ID #117509)
LEARNING OBJECTIVES
1. Recognize the criteria for diagnosing endocarditis. 2. Recognize the surgical indications
in endocarditis
CASE
A 29-year-old man presented with one week of a right buttock lesion and fevers. He
denied any past medical history or IV drug use. His temperature was 37°C, pulse 113
bt/min, respirations 32 breaths/min, blood pressure of 83/59 mmHg. With the exception
of the lesion, his exam was normal. His white count was 33,000 with 40% bands. He
was taken to the operating room for incision and drainage of the abscess and started
on vancomycin and piperacillin. The wound culture grew methacillin-resistant staph
aureus. On the third hospital day, he was noted to have Janeway lesions, a new murmur,
and chest X-ray with fluffy infiltrates. Gentamicin was added; an echocardiogram revealed
a small vegetation on the mitral valve. Blood cultures were positive for methacillin-resistant
staph aureus and remained positive despite antibiotics. CT surgery was consulted,
but declined to operate given his positive cultures. On the eighth hospital night
he complained of headache and blurred vision. A CT of the head was performed. By the
end of the CT scan, his Glasgow Coma scale had decreased to 4. The CT scan revealed
a large occipital intra-cranial hemorrhage. He was pronounced brain dead the following
morning.
DISCUSSION
While seemingly commonplace in urban hospitals, acute endocarditis carries a high
morbidity and mortality. The indications for sugery in endocarditis include acute
aortic or mitral regurgitation with heart failure, fungal infection, acute aortic
regurgitation with tachycardia and early closure of the mitral valve on echocardiogram,
and evidence of annular or aortic abscess. In these situations, surgery should not
be delayed due to persistently positive blood cultures. One of the roles of the general
internist is to advocate for the patients who cannot advocate for themselves. Internists
should be fully aware of the indications for surgery in the setting of endocarditis
to permit this advocacy where indicated.
A PAIN IN THE NECK
J.A. Kasher
1; P.P. Balingit1; A. El-Bialy1; J. Wheat1. 1UCLA San Fernando Valley Program, Sylmar,
CA. (Tracking ID #115816)
LEARNING OBJECTIVES
1. Appreciate the multiple possible clinical manifestations of aortic dissection.
CASE
A 60-year-old male with history of hypertension presented with complaint of neck discomfort
and increasing shortness of breath for 2 weeks. Initially, he felt a sudden “severe,
pressure sensation” in his jaw which radiated to his chest, neck, and back of the
head. In the next few days, the pain localized to the neck and he also developed progressive
dyspnea and orthopnea. On examination heart rate was 96, blood pressure was 171/65,
and breating rate was 30. Lungs had bibasilar rales, and heart had a diastolic murmur
best heard over the aortic area and S3 gallop. Distal extremities revealed normal,
equal peripheral pulses and moderate edema. Serum electrolytes, CBC, and EKG were
not significant. Troponin level was 1.1, but normalized later. Chest radiograph demonstrated
cardiomegaly, an unfolded aorta, bilateral pleural effusions, and pulmonary vascular
congestion. Subsequent trans-thoracic and trans-esophageal echocardiograms revealed
a type A aortic dissection involving both the ascending and descending aorta and causing
significant aortic regurgitation. The patient underwent emergent repair of aortic
dissection and was discharged shortly thereafter in good condition.
DISCUSSION
Aortic dissection is a relatively uncommon but catastrophic illness classically thought
to present with sudden-onset, unrelenting, tearing pain localized in the thorax and
radiating posteriorly. However, clinical presentations are variable, and dependent
on which areas of the aorta are involved. For instance, involvement of the coronaries
could lead to acute MI. Patient could also present with severe aortic insufficiency,
heart failure, cardiac tamponade. Involvement of the carotid or renal arteries may
lead to cerebrovascular accident or acute renal failure, respectively. Peripheral
vascular involvement may result in pulse and neurologic deficits, whereas abdominal
pain may develop with involvement of the mesenteric arteries. Type A dissection can
present as severe chest pain (79%), back pain (46%), abdominal pain (22%), syncope
(13%), CHF (9%), or cerebrovascular accident (6%). In one case series, only 64% of
patients described their pain as being sharp. Another series reports that the treating
clinician fails to initially entertain the diagnosis of aortic dissection in up to
35% of cases. Many patients later found to have aortic dissection are initially suspected
to have other conditions, such as acute coronary syndrome, non-dissecting aneurysms,
pericarditis, pulmonary embolism, aortic stenosis, or even cholecystitis. The high
mortality associated with aortic dissection makes its early diagnosis critical. This
case serves as a cautionary tale for the clinician to recognize the often “unusual”
presentations of this deadly disease, and to consider the diagnosis of aortic dissection
in any patient presenting acutely with chest or abdominal pain.
A PUZZLING CASE OF HYPOGYLECEMIA: THE CLUE IN THE MEDICATION HISTORY
S. Estes
1; M. Panda1. 1University of Tennessee, Chattanooga, Chattanooga, TN. (Tracking ID
#115171)
LEARNING OBJECTIVES
1. Recognize the importance of taking a detailed history. 2. Recognize the interactions
of herbal medications with prescription drugs.
CASE
A 30 year old white female with well controlled insulin dependent diabetes mellitus
for thirteen years presented with 2 months of numerous hypoglycemic episodes. A decrease
in her insulin regimen did not resolve the hypoglycemia. On further review of the
patient's medications, the only new addition was ginseng, which she began taking 2
months ago for “increased energy”. Complete work-up including renal function was normal.
The ginseng was discontinued and her hypoglycemia resolved. She was able to resume
her previous insulin regimen.
DISCUSSION
Herbal therapy is an ancient practice that appears to be experiencing resurgence in
the U.S. In numerous previous studies, the ginseng glycopeptides (GGP) from the roots
of Panax ginseng had hypoglycemic activity on both normal and hyperglycemic animals.
Studies in diabetic humans have also suggested that ginseng lowers blood glucose.
The hypoglycemia is due to the enhancement of aerobic glycolysis. The administration
of GGP decreases both the level of plasma lactic acid and the activities of plasma
and liver LDH while enhancing the rate limiting enzymes in aerobic glycolysis (tricarboxylic
acid cycle). The hypoglycemic action of GGP could last up to 16 hours. This case reflects
the increasing frequency of herbal and alternative medication use and supports the
fact that patients often neglect to tell their physicians. Direct inquiry about herbal
medication use should be a routine part of history taking.
A RARE CASE OF INTRATHORACIC ECTOPIC GOITER
Q. Saleheen
1; H.J. Freidman1; O. Marzouki1; S. Nizar1. 1Saint Francis Hospital, Evanston, IL.
(Tracking ID #116561)
LEARNING OBJECTIVES
1. To emphasize the need to consider ectopic goiter in the differential of a mediastinal
mass. 2. To understand that a mediastinal mass with hemorrhagic changes can cause
acute stridor. 3. To think about mediastinal mass as a cause of cough especially when
cough is positional.
CASE
A 38-year-old African American woman presented with a history of dry cough for 3 weeks
and shortness of breath with a loud noisy breathing for 1 day. Patient also complained
of generalized fatigue but no fever, no phlegm or weight loss. Patient is a non-smoker
and works as a construction worker. On examination, patient vital signs were stable
with 98% O2 saturation on room air with audible stridor. There was fullness in the
lower anterior neck but no well-demarcated mass, no lymphadenopathy or thyroid enlargement,
and no audible bruit in the neck was appreciated. The rest of examination was unremarkable.
Laboratory workup showed microcytic hypochromic anemia with Hemoglobin of 7.4. TSH,
FT4 and T3 were normal. Chest x-ray revealed a superior mediastinal mass with deviation
of trachea towards the right side. A subsequent CT scan showed a 7 cm mediastinal
mass with inhomogeneous enhancement extending from anterior to middle mediastinum
with no lymphadenopathy. Patient underwent surgical resection with removal of a cystic
mass arising from the chest beneath, but separate from the left inferior lobe of thyroid.
Both lobes of the thyroid gland appeared normal. A preliminary post-operative diagnosis
of bronchogenic cyst with tracheal compression was made. The final diagnosis was made
on biopsy, which showed benign nodular thyroid tissue with involution and hemorrhagic
changes. Patient subsequently discharged home without complications.
DISCUSSION
We present here a rare case of an ectopic intrathoracic goiter (a goiter with no attachment
to the cervical thyroid gland). Most of the ectopic goiters are reported in the neck
but rarely in the mediastinum. On review of the literature, there were sporadic cases
reported as ectopic intrathoracic goiters. A study in Germany of 61 surgically treated
intra-thoracic goiters from 1980 to 1999 showed that only 2 cases were ectopic. When
present as a mediastinal mass the ectopic goiter can cause compression symptoms in
about 40%–50% of the cases. A mediastinal goiter can cause stridor that can be gradual
in onset or sudden if there are hemorrhagic changes in goiter (as in our patient).
Also a mediastinal goiter can cause cough that can be positional in character. Thus
it is important to consider ectopic goiter in the differential of a mediastinal mass
with the evidence of airway obstruction.
A RARE CAUSE OF CIRRHOSIS IN AN EPILEPTIC
M.A. Kalpakian
1; S. Dea2. 1UCLA San Fernando Valley Program, Sylmar, CA; 2University of California,
Los Angeles, Sylmar, CA. (Tracking ID #115614)
LEARNING OBJECTIVES
1. Diagnose the etiology of cirrhosis when the cause is not obvious. 2. Review monitoring
tests for patients on antiepileptic medications.
CASE
A 31-year-old male with a history of epilepsy for over ten years presented with complaints
of nausea, vomiting, and dull left upper quadrant pain developing over the past two
months. His generalized tonic-clonic seizures and absence seizures have been controlled
with carbamazepine for ten years and valproic acid for three years. The patient did
not have diabetes or hyperlipidemia. On review of symptoms, he complained of decreased
appetite with 20 lb weight loss over the past year. The patient denied tobacco, alcohol,
or drug abuse. Physical exam was significant for a slender afebrile male with tenderness
to palpation in the left upper quadrant and hepatosplenomegaly. There was no rebound
or guarding. The patient was not jaundiced and did not have stigmata of chronic liver
disease. Labs were significant for an ALT of 104, AST of 79, alkaline phosphatase
229, INR 1.04, WBC of 3.2, Hb 12.5, Hct 37.4 and platelet count of 87. Pancytopenia
was thought to be a result of massive splenomegaly. An abdominal ultrasound showed
a liver span of 18.2 cm and the spleen was 15.2 cm × 15.3 cm with a patent portal
vein with appropriate flow. Liver biopsy revealed cirrhosis. Hepatitis B and C serologies
were all negative. Antinuclear antibody, anti-smooth muscle antibody, and antimitochondrial
antibody were negative. Iron, ferritin, iron saturation and ceruloplasmin levels were
normal. A comprehensive literature search showed no reported cases of carbamazepine-induced
cirrhosis and a few case reports of valproic acid associated with cirrhosis. Carbamazepine
and valproic acid were stopped and the patient was started on levetiracetam.
DISCUSSION
Many drugs are hepatically metabolized but drugs that induce cirrhosis are relatively
rare. Valproic acid is one of these drugs and is being used by internists for many
indications ranging from migraine headache prophylaxis to seizure disorders. Routine
monitoring of transaminases in patients on anti-epileptics is still controversial.
Some clinicians argue that since hepatic failure caused by valproic acid is an acute
idiosyncratic reaction, checking transaminases in patients who have been on valproic
acid for years may not prevent liver failure. However, as this case illustrates, chronic
liver damage and cirrhosis can result from chronic anti-epileptic drug use. A toxic
metabolite of valproic acid may be responsible for inducing non-alcoholic fatty liver
disease that may progress to cirrhosis. Perhaps monitoring transaminases every 6 months
in these patients could have diagnosed liver toxicity prior to the onset of cirrhosis.
Monitoring may have allowed this patient to be switched to another anti-epileptic
drug earlier, preventing him from developing end-stage liver disease.
A RATHER SIGNIFICANT EOSINOPHILIA
S.Y. Chien
1; A.M. Fogelman2. 1University of California, Los Angeles, Sylmar, CA; 2University
of California, Los Angeles, Los Angeles, CA. (Tracking ID #115722)
LEARNING OBJECTIVES
1. Recognize the differential diagnosis and clinical aspects of eosinophilia. 2. Distinguish
between different vasculitides.
CASE
A 43 year-old female with complicated medical history was transferred to our hospital
for six months of bilateral neck swelling and recent right-sided weakness. She reported
several other conditions that had begun in the previous 2 years including alopecia,
allergy, chronic otitis media, and whole body pruritis. She was now complaining of
a new cough, dyspnea, and exertional chest pain. Previous diagnostic work-ups found
pulmonary infiltrates, bilateral internal carotid artery aneurysms, and confirmed
a recent stroke. Her initial CBC was particularly signifcant for a WBC of 11,000 with
50% eosinophils on the differential. ESR was negative. A work up of this appreciable
eosinophilia ensued, with normal infectious cultures and rheumatologic tests (ANA
and ANCA). Because of her history of angina, a cardiac nuclear stress perfusion scan
was done, showing multiple defects with a depressed ejection fraction. Subsequent
cardiac catheterization demonstrated no significant atherosclerosis, but found aneurysmal
dilatation of all three main coronary arteries. Based on her carotid and coronary
aneurysms and significant eosinophilia, our patient was diagnosed with a vasculitis,
most likely Churg-Strauss syndrome or Takayasu's.
DISCUSSION
Eosinophilia is defined as >500 per microliter in the blood or tissue. Patients may
have multiple end-organ dysfunction, leading to thrombosis and fibrosis. Besides parasitic
or helminthic infections, other common causes are allergies, collagen vascular diseases,
and malignancies. However, the etiology of this patient's eosinophilia was due to
vasculitides, likely ANCA-associated small to medium-vessel disease (Churg-Strauss
syndrome, microscopic polyangitis, or Wegener's granulomatosis) versus large-vessel
disease (Takayasu's). It is important to realize that approximately 10% of patients
with ANCA-associated vasculitis have negative assays for ANCA. Often, there is a substantial
overlap among different vasculitides, such as in this patient. Churg-Strauss syndrome
has a characteristic triad: allergic rhinitis and asthma, systemic granulomatous inflammation
of small vessel, and virtually all patients have eosinophilia. Ofteh, it has less
renal involvement. However, coronary arteritis and myocarditis are very frequent,
accounting for major morbidity and mortality. On the other hand, Takayasu's arteritis
(aortic arch syndrome) has a strong predilection for the aortic arch and its branches.
Pulses are commonly absent, particularly if subclavian artery is involved. Although
less common, inflammation can also be found in other major arteries, including carotid
and coronary. The mainstay of treatment for most vasculitides includes corticosteroids
with or without cytotoxic drugs such as cyclophosphamide. Combined therapy induces
improvement in 90% and complete remission in 75% of patients.
A RED EYE AND VISION LOSS: NOT YOUR USUAL CONJUNCTIVITIS
T. Pestana
1; M. Landry1. 1Tulane Health Sciences Center, New Orleans, LA. (Tracking ID #117498)
LEARNING OBJECTIVES
1) Identify ocular manifestations of fungal infections. 2) Recognize risk factors
for fungal ocular infections. 3) Establish available treatments for fungal ocular
infections.
CASE
A 25 year-old woman presented with left eye pain and vision loss. She noted eye injection
and pain with movement. She also complained of lower back pain. She had fever, chills,
night sweats, and a recent fifty-pound weight loss. Her past medical history included
hepatitis C and intravenous drug abuse. Her needle-sharing companions had developed
similar ocular symptoms several weeks prior. She was afebrile, and had conjunctival,
scleral, and limbal injection with a hazy cornea, and clear ocular discharge. The
left pupil was fixed at four millimeters; visual acuity was 20/200. Direct fundoscopic
exam revealed fluffy white vitreous opacities obscuring the optic disc, and retinal
detachment. The L1–L2 right paraspinal region was tender to palpation. MRI of the
spine showed discitis, osteomyelitis, and a psoas abscess in the paraspinal region.
The patient was diagnosed with retinal detachment and fungal endophthalmitis. Intravitreal
and systemic amphotericin B were initiated. Cultures obtained from the psoas abscess
yielded Candida albicans, confirming disseminated fungal infection.
DISCUSSION
Fungal endophthalmitis is a serious infection that can lead to visual deficits. Risk
factors include intravenous drug abuse, immunosuppression, parenteral nutrition, and
ophthalmologic surgery. There is no consensus on standard antifungal treatment, but
amphotericin B, flucytosine, and fluconazole may be used. Vitrectomy may also be considered
for vision salvage. Early diagnosis, ophthalmologic evaluation, and treatment are
essential for preventing vision loss.
A RESTAURANT, AN ONION, A LIVER: A CASE OF FULMINANT HEPATIC FAILURE FROM HEPATITIS
A
P.K. Nair
1; B.S. Berk1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115944)
LEARNING OBJECTIVES
1) Recognize the epidemiology of Hepatitis A (HA), 2) Diagnose Fulminant Hepatic Failure
(FHF), 3) Manage FHF in HA.
CASE
A 57-year-old male who is on prednisone for ulcerative colitis presented with elevated
liver enzymes and change in mental status. He is a non-smoker who drinks about two
beers per week. On October 11th, he and his wife ate at a local restaurant. On October
29th, they both developed anorexia, myalgia, and diaphoresis. The patient's wife improved,
but he continued to deteriorate. On November 5th, he went to his community hospital.
His labs showed the following: ALT 6438, AST 8689, INR 3.0, and Total Bili 2.5. The
next day he became poorly responsive and was transferred to our MICU. He was deeply
jaundiced, had brisk reflexes, and right ankle clonus. Intracranial pressure was elevated.
No stigmata of chronic liver failure was noted. Labs revealed the following: glucose
50, lactate 5, ammonia 101, ferritin >5,000, ALT 3624, AST 1108, Total Bili 11.3,
PTT >100, and non-calculable PT/INR. He was promptly transferred to the Liver Transplant
ICU. Hepatitis serologies were negative for HepB, HepC, CMV, EBV, HSV, VCZ, Wilson's,
and autoimmune markers. HepA IgM was positive. The next morning he underwent orthotopic
liver transplantation. He was discharged one month later in stable condition.
DISCUSSION
There were 650 confirmed cases of HA from a recent outbreak in western Pennsylvania.
It was linked to a restaurant that served tainted green onions from Mexico. Four people
developed FHF, and only this patient survived. In the United States, there has been
a gradual decline in the number of confirmed HA cases from 1980 (29,087 reported cases)
to 2001 (10,616 reported cases). On average only 0.2% to 0.4% of cases of HA progress
to FHF. The risk of developing FHF from HA infection increases with advancing age
and in patients with chronic liver disease, especially from hepatitis C. In FHF from
HA, there is a rapid progression of severe acute liver injury with impaired synthetic
function as evidenced by decreasing AST/ALT and increasing INR. Stigmata of chronic
liver disease are absent. To be classified as having FHF one must have encephalopathy
develop within eight weeks of onset of symptoms if they had a previously healthy liver,
or within two weeks of onset of jaundice regardless of any underlying liver disease.
Management of FHF from HA should involve early referral to a liver transplant center
of high-risk patients. Transplantation improves survival rates in FHF by as much as
70% to 80%. Based on the King's College Criteria, referral for transplant of FHF from
HA should take place if the following criteria are met: 1. PT >100, irrespective of
the grade of encephalopathy, or 2. Any three of the following variables: age <10 or
>40, duration of jaundice before onset of encephalopathy >7 days, PT >50, or Total
Bili >18.
A SEVERE CASE OF ALCOHOLIC HEPATITIS
J.E. Guy
1. 1University of California, San Francisco, San Francisco, CA. (Tracking ID #117466)
LEARNING OBJECTIVES
1. Review the differential diagnosis of right upper quadrant abdominal pain. 2. Recognize
laboratory findings seen in alcoholic hepatitis. 3. Discuss treatment options for
severe cases.
CASE
A 45 year old woman with a 23 year history of significant alcohol abuse presents to
the emergency room with a several week history of nausea, nonbloody emesis, constant,
nonradiating right upper quadrant pain, and jaundice. She reports intermittent subjective
fevers. Her last drink was one day prior to admission, and she reports drinking 9
shots of brandy a week. On physical exam she was afebrile. She was somnolent but arousable.
Asterixis was present. Her abdominal exam was distended and diffusely tender to palpation,
with marked right upper quadrant pain without rebound. Her liver span was enlarged
to 12 cm but there was no fluid wave or frank ascites. Skin exam was markedly jaundice
but without spider angiomata or palmar erythema. Pertinent laboratory data included
a WBC of 41.5K, total bilirubin 17.6, AST 132, ALT 14, albumin 1.7, Cr 0.9, and PT
21.7. A CXR, urinalysis and blood cultures were negative. A right upper quadrant US
did not reveal signs of cholecystitis, obstruction or ascites. An abdominal/pelvic
CT was without evidence of cholangitis or masses. Of note, the patient was admitted
to the surgical service three weeks prior to admission with similar complaints, a
WBC of 14.8K, AST 130, ALT 20, and total bilirubin 4.6. At that time a RUQ ultrasound,
HIDA scan, abdominal CT, white blood cell scan, urine and blood cultures were within
normal limits. The patient was diagnosed with alcoholic hepatitis and told to abstain
from drinking.
DISCUSSION
This patient's clinical picture was consistent with severe alcoholic hepatitis. On
two occasions an in-depth evaluation was undertaken to evaluate for cholecystitis,
cholangitis, obstructing lesion, systemic infection or other explanations of right
upper quadrant pain, abnormal liver function tests and leukocytosis. As this case
underscores, alcoholic hepatitis manifests as an inflammatory state of the liver and
can be confused for infection or obstruction. Patients often present with right upper
quadrant pain, hepatomegaly, jaundice and fever. Hepatic encephalopathy is a poor
prognostic indicator. The mortality of the disease is high, and can be stratified
by calculating the discriminant function [formula 4.6 (PT-control) + tbili]. In this
patient with a discriminant function (DF) of greater than 32, her mortality at one
month is 50%. A small mortality benefit at two and six months has been demonstrated
with corticosteroids and at four weeks with pentoxifylline in patients with DF >32
and no evidence of infection or bleeding. This patient received pentoxifylline without
significant change in her laboratory or clinical parameters after 4 weeks of treatment.
She did survive the acute period, and in follow-up six months later, the patient had
abstained from drinking with improvement in her physical exam and laboratory values.
A SURPRISING CAUSE OF ACUTE-ONSET ALTERED MENTAL STATUS AND HYPOXIA IN A PREVIOUSLY
STABLE PATIENT
M.R. Heller
1. 1University of California, San Francisco, San Francisco, CA. (Tracking ID #115003)
LEARNING OBJECTIVES
1) Recognize air embolus as a possible cause of respiratory distress and altered mental
status in hospitalized patients. 2) Become familiar with preventative measures and
treatment options.
CASE
A 58 year old man with AML was admitted to the hospital for consolidation chemotherapy.
Three weeks into his hospital stay, he was found on the ground in the doorway to his
room, confused and unable to get up. The patient was oriented only to person, but
did not complain of any localized pain or difficulty breathing. On physical exam,
his vitals were stable with the exception of his O2 saturation, which was 84% on room
air, but increased to 97% on 10L face mask. He had no obvious signs of trauma, except
that the cap to one of the lumens of his central line was off. The remainder of his
cardiopulmonary and neurological exam was within normal limits, to the extent that
he could cooperate. Labs were unremarkable. A head CT was negative, and a chest x-ray
and CT did not show signs of new infection, pneumothorax, or pulmonary embolus. By
the following morning, the patient's altered mental status and hypoxia had resolved.
His symptoms were ascribed to an air embolus, which likely occurred during a normal
tidal volume inhalation after the cap to his central line became disconnected.
DISCUSSION
Air embolus (AE) is most commonly associated with surgical procedures, penetrating
chest injuries, barotrauma, and central venous catheterization. Slow infusions of
small amounts of air are tolerated better than large rapid boluses —it is thought
that 300–500 cc of air infused over a few seconds can be fatal in humans. Dyspnea
is found in almost all cases of AE, and may be accompanied by tachypnea, respiratory
failure, chest pain, hypotension, and tachycardia. Neurological findings are present
in up to 40% of cases, and range from dizziness and a subjective “sense of doom” to
altered mental status or focal neurological deficits. Diagnosis of AE can be difficult,
as labs, chest x-rays, VQ scans, and chest CTs are most often normal. Therefore, a
high index of suspicion is required in a patient undergoing a surgical procedure or
with central venous access. Treatment focuses on identifying the source of air entry
and preventing further air flow. Hyperbaric oxygen may be helpful in extreme cases
to reduce the intravascular air bubble size. Furthermore, positioning the patient
in the left lateral decubitus position may help to prevent an air bubble in the right
ventricle from obstructing the pulmonary outflow tract. Finally, aspiration through
the central venous catheter may be attempted if the introduction of the AE is witnessed.
Emphasis should be placed on prevention —patients should be in Trendelenburg position
and they should be instructed to valsalva or exhale during the placement or removal
of a central venous catheter.
A THORNY PROBLEM: MYCOBACTERIUM KANSASII INFECTION OF THE SKIN
K. Pachipala
1; S. Naidu1; L. Adhikesavan1; R. Gotoff1; D.R. Gutknecht1. 1Geisinger Medical Center,
Danville, PA. (Tracking ID #103207)
LEARNING OBJECTIVES
Appreciate that M.kansasii can cause water-borne skin infections.
CASE
A 43-year-old woman developed erythema and swelling over a PIP joint of her left hand
three months after cutting that finger on a thorn. Pus was aspirated but no bacteria
were found on gram stain or culture. Subsequent biopsy showed a granulomatous reaction
and cultures grew pigmented mycobacteria. The patient had a history of exposure to
both an inground swimming pool and a hot tub and was empirically treated with Bactrim
for presumed infection with M.marinum, since that agent is the commonest pigmented
mycobacterium causing skin infections. The organism was later identified as M.kansasii
and the patient was given INH, rifampin and ethambutol. INH and rifampin could not
be continued because of hepatotoxicity, and after consultation with a national expert,
an alternative treatment regimen of clarithromycin, gatifloxacin and ethambutol was
instituted and the patient improved.
DISCUSSION
M.kansasii is a slow growing, photochromogenic mycobacterium found in potable water
supplies, swimming pools and sewage. Cutaneous M.kansasii infections are rare, with
44 cases so far reported in the literature. These infections are sporadic and usually
due to inoculation following minimal cutaneous trauma. Most occur in patients with
immunological problems, and patients may present with papules, nodules, pustules,
crusted ulcers, cellulitis or sporotrichial lesions. The American Thoracic Society
recommends treatment with INH, rifampin and ethambutol. In our patient the history
suggested both thorn injury and water exposure as possible vectors of infection. Thorn
prick is usually associated with infections with gram-negative bacteria, clostridial
species or sporotrichosis. Bacteria associated with water exposure include Aeromonas
sps, Edwardsiella tarda, Erysipelothrix rhusiopathiae, Vibrio vulnificus and Mycobacterium
marinum. The surprise finding of M.kansasii confirmed this was a water-borne infection,
and an unusual one at that.
A VERY RARE PRESENTATION OF A COMMON ILLNESS, INFLUENZA A INFECTION WITH A MASSIVE
PERICARDIAL EFFUSION
S.G. Khurshid
1; U. Ahmed2; P. Sherchan3. 1Saint Francis Hospital at Evanston, Evanston, IL; 2Saint
Francis Hospital, Evanston, IL; 3Saint Fancis Hospital at Evanton, evanston, IL. (Tracking
ID #115934)
LEARNING OBJECTIVES
To recognize an unusual complication of Influenza A virus.
CASE
21-year-old previously healthy male presented with seven days history of pleuritic
chest pain and fever. Chest pain was persistent and worsened with deep breathing and
lying down. Patient also described a recent upper respiratory illness associated with
fever, chills and myalgias. Physical examination revealed a temperature of 100.5 F,
pulse rate of 111, BP of 105/65 and respiratory rate of 18. The heart sounds were
distant with a prominent pericardial rub. Decreased breath sounds and dullness were
present at the left lung base. No rales were audible, liver was not enlarged and there
was no peripheral edema. No jugular venous pulsation was visible and pulses paradoxus
could not be detected. Lab workup revealed WBC count of 11.4 with 40 percent lymphocytes.
Serological screening for viruses including CMV, Coxsackie, EBV, VZV, HIV and Echovirus
was negative. Rapid influenza antigen detection from nasophyrengeal swab was positive
for influenza A virus. EKG revealed sinus tachycardia with no evidence of electrical
alternans. CXR showed cardiomegaly with bilateral pleural effusions. CT scan of the
chest showed a fluid collection in the pericardium with abnormal enhancement of the
pericardial lining indicating pericardititis. Echocardiogram revealed massive pericardial
effusion with no evidence of cardiac tamponade. Patient improved with NSAIDs, oseltamivir
and fluid resuscitation.
DISCUSSION
Influenza A and B infections are recognized causes of pericarditis. Viral pericarditis
may be associated with myocarditis and in this case, the pericardial inflammation
was sufficient to cause a massive effusion. Myocarditis and pericarditis were reported
in association with influenza viral infection during the 1918–1919 pandemic; these
reports were largely based on histopathologic findings, and these complications have
been reported only infrequently since then. To our knowledge only a few cases of a
massive pericardial effusion causing tamponade secondary to influenza have been reported
but all those patients had underlying cardiac disease. This case appears to be unique
in that the presence of massive pericardial effusion did not cause clinical or echocardiographic
evidence of tamponade. We attribute this to the young age and normal underlying myocardium.
ACQUIRED FACTOR VIII INHIBITOR PRESENTING AS REFRACTORY GI BLEEDING
R.R. Yeldandi
1; M. Peek1. 1Rush University/Rush- Presbyterian-St. Luke's Medical Center, Chicago,
IL. (Tracking ID #116937)
LEARNING OBJECTIVES
(1) Recognize underlying coagulopathies as etiologies of refractory GI bleeding. (2)
Understand the treatment options for acquired factor VIII inhibitors.
CASE
The patient is a 49 year old woman with a history of hypertension, coronary artery
disease, and CHF who presented after two episodes of hematemesis; she denied hematochezia
or melena. She had no history of peptic ulcer disease, dyspepsia or NSAIDs use. Nasogastric
lavage yielded 500 ccs of coffee grounds and her stool was heme positive. An emergent
EGD revealed a gastric ulcer whose actively bleeding vessel was coagulated with electrocautery.
She was started on Protonix. Three days later, the patient had hematochezia and hematemesis.
Repeat EGDs showed an adherent clot but no active bleeding. H. pylori titers and a
colonoscopy were negative. With no other source of active bleeding identified, the
gastric ulcer was surgically resected, but the patient had persistent post-operative
bleeding. On admission, the patient had a prolonged aPTT (>240 sec) which was previously
normal; aPTT mixing studies were positive for a coagulation factor inhibitor. Factor
VIII inhibitor levels were elevated, thus diagnosing the patient with an acquired
factor VIII inhibitor coagulopathy. The patient was treated with Factor Eight Inhibitor
Bypass Activator (FEIBA), recombinant activated factor VIIa, steroids and Imuran.
The patient was discharged in stable condition.
DISCUSSION
Factor VIII inhibitor is an autoantibody directed against coagulation factor VIII.
Patients can present with hematomas, hematuria, GI bleeding, retropharyngeal or retroperitoneal
bleeding, cerebral hemorrhages and/or post-operative bleeding. Recombinant factor
VIIa infusions can activate the coagulation cascade by bypassing factor VIII and control
acute bleeding. Maintenance therapy with immunosupressants can lower inhibitor titers.
Our patient's acute bleeding resolved with infusions of FEIBA and recombinant factor
VIIa. Although she presented with a common clinical manifestation of factor VIII inhibitor,
the etiology of her uncontrolled bleeding was uncommon. Our patient had a clear source
of GI bleeding, but she did not respond to standard treatment. Without her underlying
coagulopathy, her bleeding may have ceased with cautery. We present this case in order
to bring attention to possible uncommon etiologies for common scenarios. Failure of
conventional therapy for GI bleeding should prompt further evaluation for an underlying
coagulopathy. Early identification of acquired coagulopathies and treatment with effective
agents can reduce excessive blood loss and reduce mortality.
ACTINOMYCOSIS PRESENTING AS A SKIN ABSCESS
J. Blank1; M. Traina
1. 1UCLA— San Fernando Valley Program, Sylmar, CA. (Tracking ID #116705)
LEARNING OBJECTIVES
1) Recognize Actinomyces as a cause of skin abscess. 2) Discuss the diagnosis and
management of disseminated Actinomycosis.
CASE
A 46 year old male presented to the clinic with a three month history of left shoulder
pain and progressive loss of range of motion. The patient noticed a lump on his shoulder
6 weeks ago that has been increasing in size. He admits to a productive cough, dyspnea
on exertion, and fatigue. The patient smokes 2 packs per day, drinks 6 beers per day,
and works as a repairman, often underneath houses. Physical examination revealed a
fluctuant 13 × 13 cm mass over the left scapula, poor dentition, and rhonchi over
the left lung base without evidence of lymphadenopathy. Significant laboratory results
included a WBC 12.9, Hemoglobin 7.8, and Folate 2.7. Chest x-ray showed a nodule in
the left lower lobe. CT scan of the chest revealed a 3 cm irregular soft tissue mass
in the left lower lobe with apparent sinus tracts extending to the back. Fine needle
aspiration of the shoulder mass was completed and Gram stain demonstrated “sulfur
granules” consistent with Actinomyces infection. After surgical drainage of the abscess,
intravenous ampicillin-sulbactam was initiated for 4 weeks followed by oral penicillin.
Repeat CT scan after 4 weeks of treatment revealed a decreased lung mass size.
DISCUSSION
Actinomycosis in humans is commonly caused by Actinomyces israelii. It frequently
occurs in adult males with dental infections but also can occur in patients with diabetes,
immunosuppression, malnutrition and local tissue damage with neoplastic disease or
irradiation. Actinomycosis generally arises from a dental source but in rare cases
it can originate from the thyroid gland, thyroidectomy incision sites or lung as seen
with this patient. Typically, infection spreads by direct invasion without respect
to anatomical barriers including fascial planes, forming multiple abscesses. Pulmonary
infections usually arise after aspiration of oropharyngeal or gastrointestinal secretions.
The most common pulmonary clinical presentation is an indolent, progressing pneumonia
with or without pleural involvement. Patients present with a productive cough, fever,
chest pain, and weight loss. Actinomyces is difficult to culture and should be suspected
when “sulfur granules” are seen on visual or microscopic examination. Sulfur granules
which are rarely seen with Nocardiosis, are common with Actinomycosis and have a characteristic
cauliflower-like appearance upon microscopic examination. Abscesses require surgical
drainage and the infection is highly responsive to antibiotic treatment with long
term penicillin.
ACUTE ONSET GENERALIZED LYMPHADENOPATHY IN A 58 YO MALE
B.E. Gewurz
1; J. Beach2. 1Beth Israel Deaconess Medical Center, Boston, MA; 2Beth Israel Deaconess
Medical Center, Brookline, MA. (Tracking ID #115887)
LEARNING OBJECTIVES
Distinguish between local and generalized adenopathy. Diagnose etiology of generalized
lymphadenopathy. Recognize when a lymph node biopsy is indicated.
CASE
Mr. JW is a 58 yo male with no significant PMHx who presented to an urgent care visit
with a chief complaint of “bumps” on his neck of several days duration. He denied
associated symptoms, including fevers, chills, night sweats, cough, myalgia or arthralgia.
He denied any recent travel, sick contacts, or unusual exposures at the supermarket
that he manages. He takes no medications. He has been married for 30 years, has a
remote smoking history, and denied IV drug use. He has a pet cat. Physical examination
was notable for numerous 1–3 cm nontender, firm, mobile lymph nodes in the preauricular,
postauricular, occipital, anterior and posterior cervical, axillary and inguinal chains.
There was no hepatosplenomegaly, rashes, or mucosal lesions. An extensive work-up
did not reveal likely infectious causes of adenopathy: PPD was negative and serologic
tests failed to show acute infection by HIV-1, CMV, EBV, toxoplasma, syphilis, borrelia,
or bartonella. No HIV RNA was detected. ESR was 16 mm/hr. No acute cardiopulmonary
process was evident on chest film. CBC revealed WBC of 5,000 cells/uL, HCT of 40.8%,
and PLT of 282,000 cells/uL. Regressing adenopathy was noted at a subsequent visit,
with interval onset of night sweats, low grade fever, 5-lb weight loss over two weeks,
fatigue, persistent dry cough and generalized pruritis. A chest CT scan revealed striking
systemic adenopathy of all major nodal groups. Submandibular biopsy revealed features
consistent with angioimmunoblastic T-cell lymphoma (AILD). Bone marrow biopsy revealed
AILD involvement.
DISCUSSION
Peripheral lymphadenopathy may be the only sign of an underlying systemic process.
We review the importance of recognizing regional versus generalized adenopathy, their
differential diagnoses, and studies that should be considered. Generalized adenopathy
signifies a serious associated condition and that requires further evaluation. When
should a lymph node biopsy be obtained? Although many patients with adenopathy fear
a diagnosis of cancer, adenopathy often results from an infectious illness and only
rarely represents malignancy in the primary care setting. The need for definitive
diagnosis should be weighed against the morbidity of an invasive procedure. We discuss
circumstances where an observation period is a safe alternative and review algorithms
that predict the appropriateness of lymph node biopsy.
ACUTE RENAL FAILURE CAUSED BY A RARE BLEEDING COMPLICATION OF ENOXAPARIN
M. Bandara
1; B.P. Sankarapandian1; S.K. Thambidorai1; S. Dhanireddy2; D. Schuller1. 1Creighton
University, Omaha, NE; 2Creighton University Medical School, Omaha, NE. (Tracking
ID #115704)
LEARNING OBJECTIVES
1. Recognize the potential for enoxaparin induced intra-abdominal bleed 2. Identify
acute renal failure as a potential secondary complication of intra-abdominal bleed
3. Assess causes for post obstructive uropathy.
CASE
A 43-year-old white female with recent history of multiple upper respiratory tract
infections presents to the emergency room with dyspnea. She was found to be in atrial
fibrillation with rapid ventricular response. The patient was admitted and treated
with enoxaparin and diltiazem infusion. She was subsequently cardioverted to normal
sinus rhythm using direct current cardioversion. During the hospitalization she developed
left lower quadrant abdominal pain with associated oliguria. She rapidly progressed
to anuria prior to the obtaining of urine studies. Renal ultrasound showed bi-lateral
hydronephrosis. Urology performed a cystoscopy, which showed a compression of the
bladder from an external source. Bi-lateral ureteral stents were placed extending
from the renal pelvis into the urethra. Post operatively the patient became severely
hypotensive requiring multiple transfusions and vasopressor support. Computerized
tomography (CT) of the abdomen showed a massive hematoma in the pelvis surrounding
and compressing the bladder. The patient remained anuric for several days requiring
continuous venous-venous hemodialysis. Her renal function eventually improved and
hemodialysis was discontinued.
DISCUSSION
Enoxaparin is a rare cause for intra-abdominal bleed. This patient presented with
obstructive uropathy resulting in acute renal failure. The initial cause for the obstruction
was not apparent. Evaluation of the renal failure led to the discovery that the patient
had extrinsic bladder compression from a massive intra-pelvic hematoma. The severity
of the bleeding led to a prolonged state of renal hypoperfusion, which resulted in
acute tubular necrosis. In patients where the cause of obstruction is not readily
apparent, one must consider other potential factors. Post-obstructive renal failure
is commonly due to tumors, prostatic hypertrophy, calculi, and strictures. This case
demonstrates that pelvic bleeding should also be included in the differential diagnoses
of post-obstructive renal failure in patients receiving anti-coagulation therapy.
ADRENAL INCIDENTALOMA, CUSHING's SYNDROME, AND INSULIN RESISTANCE SYNDROME
M.N. Phan
1; N. Mikhail1; M. Rotblatt1. 1UCLA SFVP-Olive View Medical Center Department of Internal
Medicine, Sylmar, CA. (Tracking ID #117285)
LEARNING OBJECTIVES
1. Recognize an approach to diagnosing adrenal incidentaloma. 2. Be aware of the possibility
of Cushing's syndrome in adult patients with an insulin resistance syndrome.
CASE
A 51 year old male was referred for further evaluation and management of an adrenal
incidentaloma found on abdominal CT, which was originally performed to rule out nephrolithiasis.
There were no kidney stones, but instead, a 3 cm left adrenal mass was reported. His
PMH included hypertension for 6 years and diabetes mellitus for 3 years. The patient
did not report easing bruising, headache, sweating, or palpitations. Review of systems
was significant for weight gain of 50 lbs over 5 years. Family history was also positive
for diabetes and hypertension. Vitals signs were T37, BP 181/90, P115, RR16, and WT
296 lbs. Physical exam was significant for obesity, moon facies, and supraclavicular
fat pads, but no buffalo hump or abdominal striae. Cushing's syndrome was suspected,
and laboratory investigation revealed an elevated 24-hour urine cortisol of 134.2
ug/24 hrs (4–50), and suppressed ACTH of <5 PG/ML (15–70). Serum renin and aldosterone
were normal, as were urine catecholamines. The patient was diagnosed with Cushing's
syndrome secondary to an adrenal mass and was subsequently referred for adrenalectomy.
DISCUSSION
The incidental discovery of an adrenal mass is not an uncommon event due to the routine
use of common imaging techniques. The prevalence of adrenal incidentaloma is about
2.3 % at autopsy and 0.5–2% by abdominal CT scan. Hyperfunctioning develops in 1.7%
of cases, and the risk is higher in patients with lesions larger than 3 cm, of which
cortisol hypersecretion is the most likely disorder. Evaluation of an adrenal incidentaloma
includes hormone studies to determine whether the patient has pheochromocytoma, glucocorticoid
excess, primary aldosteronism, or virilizing or feminizing tumors. This is especially
important if signs and symptoms of hormonal excess are present. Cortisol secreting
adrenal incidentaloma has been implicated in causing obesity, glucose intolerance/type
2 diabetes, hypertension, and dyslipidemia or the insulin resistance syndrome. Screening
for Cushing's syndrome in this patient population, i.e., those with larger masses
and/or potential signs or symptoms of hormonal hypersecretion, can be critical. Adrenalectomy
should ameliorate insulin resistance as well as the vascular risk profile of these
patients.
ADULT PRESENTING WITH A PEDIATRIC DISEASE
M. Bandara
1; B.P. Sankarapandian1; S.K. Thambidorai1; S. Dhanireddy2; J. Derby1. 1Creighton
University, Omaha, NE; 2Creighton University Medical School, Omaha, NE. (Tracking
ID #115703)
LEARNING OBJECTIVES
1. Identify the clinical manifestations of Henoch-Schonlein purpura. 2. Recognize
the organ systems affected by the disease. 3. Demonstrate that pediatric diseases
can present in adulthood.
CASE
A 21 year old white male with a 4 month history of bloody diarrhea and poly-arthritis
presents to an outpatient clinic with pain, stiffness, swelling and purplish discoloration
of the right ankle. These skin changes progressed to multiple purpuric lesions over
the bilateral lower extremities. Erythrocyte sedimentation rate, urine analysis, rheumatoid
factor, human leukocyte antigen B-27 and radiologic studies of the ankles were all
normal. Skin puncture biopsy of these lesions showed evidence of leukocytoclastic
vasculitis. IgA antigen was positive on the vascular wall confirming the diagnosis
of Henoch-Schonlein purpura (HSP). The patient was then started on oral prednisone
therapy. He responded well to the steroids, and they were subsequently tapered.
DISCUSSION
HSP is a subtype of Leukocytoclastic vasculitis which affects multiple organ systems.
Skin involvement is seen in virtually all patients, manifesting as a palpable purpura
in the lower extremities and the buttocks. Polyarthralgias are also a common clinical
manifestation. Gastrointestinal symptoms are characterized by colicky abdominal pain,
vomiting, diarrhea, and the passage of blood. Renal involvement is seen in almost
80% of the patients and has a nephritic urine sediment and moderate proteinuria. The
presence of mesangial IgA on immunofluorescence microscopy is diagnostic. Less than
10% of the patients will progress to chronic renal failure and persistent hypertension.
HSP has the highest incidence in children with a median age group of 5 years. Classic
skin manifestations occurring early in the disease help diagnose HSP in the pediatric
age group. Our case presented with bloody diarrhea demonstrating the need for physicians
to be cognizant that HSP may initially manifest in atypical organ systems in adult
populations. Steroids are the first line of treatment for this disease.
ALCOHOLIC HEPATITIS—A CAUSE OF FEVER OF UNKNOWN ORIGIN
M. Gaddamanugu
1; F. Salahauddin1; M. Aiyer2. 1University of Illinois at Peoria, Peoria, IL; 2University
of Illinois at Chicago, Peoria, IL. (Tracking ID #117117)
LEARNING OBJECTIVES
Identify alcoholic hepatitis as one of the causes of fever of unknown origin. Recognize
the classic presentation of alcoholic hepatitis Discuss the diagnostic work up of
fever in a patient presenting with alcoholic hepatitis.
CASE
A 52-year-old female with significant history of alcohol and IV drug abuse presented
with 3-week history of jaundice, fever, and malaise. Past medical history was significant
for hypertension and asthma. Review of systems was remarkable only for a 30 lb weight
loss. Initial evaluation revealed markedly elevated liver enzymes, and anemia. Blood
cultures, urine cultures and chest radiographs were normal. The diagnosis of alcoholic
hepatitis was entertained. However patient was persistently febrile even 10 days after
hospital admission. Exam revealed a cachetic female with marked scleral icterus. Vital
signs showed temp 102.3 F, B.P 98/52, RR 20. Abdominal exam revealed hepatosplenomegaly
with minimal ascites. Patient underwent an extensive workup including CT scan of abdomen,
pelvis and chest, gallium scan, bone marrow studies and temporal artery biopsy, all
of which were normal. Her ANA, HIV and hepatitis panel were negative. Liver biopsy
revealed alcoholic steatohepatitis. Over the course of 6 weeks of hospital stay, patient
gradually deferveresed with supportive treatment and was discharged in stable condition
DISCUSSION
Malaise, fever, jaundice and tender hepatomegaly represent the classic syndrome of
alcoholic hepatitis. However, the full syndrome occurs only in a minority of patients
with alcoholic liver disease. In addition, the fever in alcoholic hepatitis is very
modest usually less than 101 degree Fahrenheit. High temperatures warrant a work up
for alternative diagnosis. This case represents a patient who presented with the classic
syndrome of alcoholic hepatitis and persistent high fever all attributed to alcoholic
hepatitis. Alcoholic hepatitis can be considered as a cause for fever of unknown origin.
ALTERED MENTAL STATUS AND AN ABDOMINAL MASS
L.B. Chun
1; R.V. Liddicoat1. 1Massachusetts General Hospital, Boston, MA. (Tracking ID #115932)
LEARNING OBJECTIVES
1) To recognize the clinical manifestations of unmanaged renal failure 2) To understand
the complications of decompressing a massively distended bladder
CASE
A 59 year old male presented with three months of nausea, altered mental status, difficulty
urinating, and a 30lb weight loss. Physical exam revealed a pale, cachectic man with
slowed speech, fetor uremicus, and a midline 20 × 20 cm, hard, nontender abdominal
mass. Labs revealed a hematocrit of 15, potassium 5.0, HCO3 8.6, BUN 243, creatinine
17, phosphorus 8.2 and iCa 0.81, and a reticulocyte count of 2.1. His abdominal CT
revealed a markedly distended bladder, severe hydronephrosis, and an enlarged prostate.
A foley catheter was placed and 1.7L of blood tinged urine was drained. A renal ultrasound
after bladder drainage showed a 10 × 10 cm bladder mass and moderate hydronephrosis
with no significant cortical thinning. Cystoscopy revealed over 4 units of malodorous
blood clot in the bladder, a normal sized prostate, and markedly friable bladder walls.
The clots were removed and bilateral ureteral stents placed. The patient continued
to bleed from the bladder despite the use of desmopressin acetate and required 14
units of packed red blood cells over 9 days to increase his hematocrit from 15 to
32. Despite expedient removal of the patient's obstruction, the patient did not regain
full renal function and required dialysis.
DISCUSSION
This case illustrates an unusual cause of renal failure as well as many of its complications.
It also demonstrates the problems that can arise from decompression of a massively
distended bladder. The electrolyte abnormalities commonly seen in untreated renal
failure include uremia, hyperkalemia, metabolic acidosis, and hyperphosphatemia. Nausea,
altered mental status, anorexia, and fetor uremicus are classic signs of uremia. High
levels of blood urea nitrogen can also inhibit platelet action and lead to excessive
bleeding. In addition, hypocalcemia and anemia can result from decreased 1,25- OH
vitamin D and erythropoetin production. Acute decompression of the bladder can result
in bleeding through re-expansion of previously compressed bladder wall veins. This
is difficult to ameliorate through intermittent clamping. Reflex vasodilation occurs
with bladder decompression leading to hypotension, particularly in hypovolemic patients.
Although it has been suggested that more gradual bladder decompression can reduce
hypotensive episodes, this is of unproven efficacy. Post-renal causes of acute renal
failure account for 5–15% of cases of renal failure cases, the vast majority due to
BPH. Early recognition of renal failure secondary to obstruction and prompt relief
of the obstruction are important in preventing permanent renal damage. Normal urine
output does not rule out obstructive causes of renal failure. Most recovery of renal
function occurs within 7–10 days after relief of the obstruction.
AN ESSENTIAL CASE OF NON-ESSENTIAL HYPERTENSION
M. Guidry
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117468)
LEARNING OBJECTIVES
1. Recognize the symptoms and signs that suggest secondary hypertension 2. Diagnose
adrenal adenoma using clinical and radiographic criteria
CASE
A 31-year-old man presented with five days of shortness of breath. His baseline dyspnea
on exertion increased from shortness of breath at five blocks, to walking 30 feet.
His one-pillow orthopnea increased to sleeping for minutes at a time sitting straight
up in bed; he developed new onset paroxysmal nocturnal dyspnea and pedal edema. The
shortness of breath was associated with a cough productive of clear sputum and fatigue.
He has a past history of hypertension and systolic dysfunction. His out-patient medications
included clonidine, metoprolol, and K-dur 40 meq. His blood pressure was 195/126 mmHg,
pulse 80 beats/min, respiratory rate 26 breaths/min, and temperature 38.3°C. He had
eight cm of JVD, an S3 gallop, and bibasilar crackles. Despite his supplementation,
his potassium was 2.7 mg/L. He had LVH on EKG and bilateral cephalization on chest
X-ray. He was admitted with the diagnosis of a CHF exacerbation and treated with diuresis
and blood pressure control. The inability to control his blood pressure despite several
medications and the continuing potassium supplementation requirements in the absence
of a diuretic prompted an evaluation for the diagnosis of primary hyperaldosteronism.
This was confirmed with a serum aldosterone of 21.3 (normal <16) and a rennin level
of <0.8. An abdominal/pelvic CT showed an 18 × 14 mm round adrenal mass with central
attenuation.
DISCUSSION
Primary hypertension is so common as to mask the red flags of secondary hypertension.
A search for secondary causes of hypertension should be initiated when faced with
any of the following: hypertension in youth, hypertension requiring multiple medications,
episodes of flash pulmonary edema or unexplained congestive heart failure, hypertension
with unexplained electrolyte abnormalities, and any of the above coupled with an incidentaloma
found on CT. The patient in this case presented was diagnosed with hypertension at
the age of 26, and his blood pressure was poorly controlled while being treated with
multiple medications. He required potassium supplementation despite not being on potassium-wasting
medications. He was hospitalized twice for unexplained congestive heart failure and
had a benign adenoma was discovered on abdominal CT. Following the removal of his
adrenal adenoma, his hypertension resolved.
AN INTERESTING CASE OF HYPERTHYROIDISM IN PREGNANCY
N. Mehta
1; R.O. Powrie2; L. Larson2; K. Rosene-Montella2. 1Women and Infants' Hospital, Providence,
RI; 2Brown University, Providence, RI. (Tracking ID #116673)
LEARNING OBJECTIVES
LEARNING OBJECTIVES: 1) Correctly interpret thyroid function tests (TFTs) in pregnancy
2) Review the relevant differential diagnosis and management of hyperthyroidism in
the pregnant patient.
CASE
We were asked to consult on a 22-year-old woman at 18 weeks gestation for elevated
blood pressure and abnormal TSH. She had been healthy until 3 months prior to presentation
when she first noted increasing lower extremity edema. She had also noted blurry vision
and headache in the preceding week. In the emergency room, she was noted to be tachycardic
(102/min) and hypertensive (190/110 mmhg). Her physical exam was unremarkable except
for a fine tremor of the hands and 3+ pitting pedal edema. A urine dipstick revealed
3+ proteinuria. Lab abnormalities were consistent with preeclampsia. A TSH level was
found to be <.01 U/ml. Her full thyroid function panel was consistent with hyperthyroidism.
She was admitted with a diagnosis of thyrotoxicosis and severe preeclampsia at early
gestational age. A fetal ultrasound revealed multiple congenital anomalies consistent
with triploidy. The patient chose to terminate the pregnancy. Placental pathology
was consis-tent with a partial hydatiform mole. Postpartum, the patient's tachycardia,
hypertension and tremor resolved within a day. A -hCG level on the third postpartum
day was still elevated at 42,700 u/ml. At her 6-week postpartum check, the patient
was well, with normal thyroid function tests and -hCG level <5 u/ml.
DISCUSSION
Hyperthyroidism is the second most common endocrine problem encountered in pregnant
women. Recent evidence has emphasized the importance of a euthyroid state in pregnancy
for favorable maternal and fetal outcomes. Internists should be prepared to diagnose
and manage thyroid disease in pregnancy. Changes in serum concentrations of thyroid
hormones and thyroxine-binding globulin during pregnancy make the interpretation of
TFTs in pregnancy difficult. The expected changes in TFTs with each trimester and
their relationship to hCG levels will be dis-cussed. The pathophysiology, clinical
presentation and management of hyperthyroidism in pregnancy will be discussed. Typical
causes of hyperthyroidism in pregnancy, including Grave's disease and Hashimoto's
thyroiditis will be reviewed and differentiated from hyperthyroidism associated with
gestational trophoblastic disease and hyperemesis gravidarum.
AN OVERLOOKED DIAGNOSIS IN THE ELDERLY
C.L. Cullinane
1. 1Boston Medical Center, Boston, MA. (Tracking ID #116564)
LEARNING OBJECTIVES
1. To enhance clinician awareness of the need for HIV testing in the elderly. 2. To
recognize the importance of a complete history. 3. To recognize the need for further
research on HIV prevention and treatment among the elderly.
CASE
A 77 year old Cape Verdean-male was admitted with trigeminal zoster. Past medical
history was significant for hypertension, pneumonia with sepsis, recurrent urinary
tract infections, anemia of chronic disease, malnutrition, ischemic cardiomyopathy,
and renal insufficiency. He was a nonsmoker and denied IVDU or prior transfusions.
His wife had recently died of unknown causes. Three years prior to admission, a persistently
elevated total protein prompted an evaluation for multiple myeloma. Bone marrow biopsy
revealed a polyclonal gammopathy and a skeletal survey was negative. Subsequently
a leukemia and lymphoma panel was unremarkable. Prior to admission he was being followed
by a hematologist for Monoclonal Gammopathy of Undetermined Significance. As an inpatient
with trigeminal zoster he tested positive for HIV with a CD4 count of 70 and a viral
load of 38,386, suggesting advanced disease. HAART therapy was instituted after discharge
with suppression of his viral load. He died of an arrhythmia almost 2 years later
in the setting of decompensated heart failure.
DISCUSSION
Individuals over 50 years of age account for up to 10% of AIDS cases reported to the
CDC, a number that is expected to rise as a result of improved survival of patients
with treated disease. Older adults are less likely to use a condom during sexual intercourse
or to participate in HIV testing. Older adults with HIV infection are more likely
to be diagnosed late in disease due to delayed recognition, they experience progression
more quickly, and they survive for shorter periods of time than their younger counterparts.
Co-morbidities often complicate management and controlled data on tolerability and
responses to HAART are lacking. The possibility of HIV infection must be considered
among elderly patients with clinical features of immunodeficiency in order to avoid
delay in counseling and treatment. This case emphasizes the importance of conducting
the sexual history, regardless of age, and it underscores the need for age-appropriate
prevention and treatment strategies.
AN UNCOMMON CAUSE OF CIRRHOSIS, OR IS IT?
S. Evans
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117456)
LEARNING OBJECTIVES
1. Recognize the complications of jejeunoileal bypass prior to the onset of liver
failure 2. Recognize the presentation of vitamin deficiency.
CASE
A 62 year-old white woman presented with a two-year history of intermittent jaundice
and a three-week history of increasing abdominal girth. She had a history of jejeunoileal
bypass in 1978 for morbid obesity. She had been admitted fourteen months prior with
a hemoglobin of 4 g/dl, an elevated bilirubin, and a prolonged prothrombin time. She
was diagnosed with B12 deficiency and malabsorption of fat soluble vitamins. A CT
at that time showed diffuse fatty infiltration of the liver. On exam she had peripheral
wasting, spider angiomas, shifting dullness, jaundice, and peripheral edema. On admission,
she had a prolonged prothrombin time, a normal hemoglobin, an elevated bilirubin,
and low cholesterol levels. An abdominal CT revealed large volume ascites and a cirrhotic
liver. Paracentesis was consistent with portal hypertension as an etiology for the
ascitic fluid. A liver biopsy showed severe steatosis with cirrhosis.
DISCUSSION
Jejeunoileal bypass has been a common treatment for morbid obesity, but has rapidly
lost favor due to its severe long term consequences, including arthritis, B12 deficiency,
cirrhosis, and chronic diarrhea. Patients who received this procedure are now coming
to the attention of physicians because of cirrhosis. Unfortunately, this patient was
regularly followed in the medicine clinic but did not undergo hepatic evaluation because
her symptoms were attributed to the altered physiology of bypass. The intermittent
episodes of jaundice following the correction of the B12 deficiency suggested another
underlying pathology. Similarly, the prolongation in the prothrombin time following
replacement of vitamin K should have been a clue that there was impaired hepatic synthetic
function. The CT scan of the abdomen was also potentially confusing: because of the
morbid obesity, hepatic steatosis was attributed to NASH. Prompt recognition of this
complication of jejeunoileal bypass is important to refer patients to a hepatologist
to prevent the expected fifty percent mortality after the development of ascites.
At present our patient is awaiting liver transplant.
AN UNEXPECTED CAUSE OF TREMOR AND MYOCLONUS
K. Nashar
1; E. Anish1; N. Busis1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116914)
LEARNING OBJECTIVES
1. To recognize Creutzfeldt-Jakob Disease (CJD) as a cause of tremor and myoclonus.
2. To appreciate the value of performing a brain biopsy to confirm a diagnosis of
CJD.
CASE
A 70 year-old female presented with a 6-week history of abnormal motor movements.
Initially, she developed an action tremor in the right arm. After 4 weeks, the left
arm also became affected and she began to experience myoclonus in both arms. On physical
exam, the patient appeared well-nourished and was afebrile. Her Mini-Mental Status
Exam score was 30/30. CNs II-XII were intact. Action-induced myoclonic jerks were
noted in both arms (R > L). Strength was 5/5 in all extremities. Sensory testing was
normal. DTR's were +2 and symmetric. Romberg test was negative. Cerebellar testing
was normal. A brain MRI was performed revealing “gyriform” signal changes in the parietal-occipital
region bilaterally. This raised suspicion for a diagnosis of encephalitis. As a result,
a lumbar puncture was performed that showed no evidence of pleocytosis or increased
protein. Over the next 4 weeks, the patient developed worsening neurological changes,
including: dysarthria, ataxia, and increased myoclonus. A repeat brain MRI was unchanged.
An EEG was normal. Serological studies looking for evidence of an autoimmune disease
were negative. Anti-Hu and anti-Yo antibodies were negative. Since a diagnosis remained
elusive and the patient's clinical condition continued to deteriorate, a brain biopsy
was performed. The pathology revealed spongiform changes consistent with CJD. The
patient's neurological impairment progressed, including the development of dementia,
and she died 2 weeks later.
DISCUSSION
CJD is a degenerative disease of the central nervous system that is caused by infectious
proteins called prions. Various forms of CJD have been recognized including: sporadic,
familial, iatrogenic, and new-variant CJD. Most patients with CJD have the sporadic
form and present with some degree of cognitive impairment and tend to progress rapidly
to a state of dementia. A myriad of other neurological signs and symptoms may develop
throughout the course of the illness with >90% of patients developing myoclonus. A
typical clinical course may suggest CJD, but confirming this diagnosis can be challenging.
Blood and CSF analyses can help exclude other conditions that may mimic CJD. Most
patients demonstrate characteristic periodic complexes on EEG and/or abnormal signal
patterns on MRI, but these findings are nonspecific. The gold standard for diagnosis
remains brain biopsy. This case is unique in that cognitive impairment did not develop
until extremely late in the course of the illness. It also emphasizes how an atypical
clinical presentation, the absence of the more common EEG and/or MRI abnormalities,
and a lack of risk factors for prion disease, can result in the delay of a diagnosis
of CJD being made.
AN UNUSUAL CASE OF ACUTE SICKLE HEPATIC CRISIS
D. Zell
1; E. Choe1; D. Spruill1. 1Tulane Health Sciences Center, New Orleans, LA. (Tracking
ID #117441)
LEARNING OBJECTIVES
1. Recognize the common and uncommon abnormal laboratory findings associated with
sickle cell disease. 2. Recognize hepatitis as a complication of vaso-occlusive crisis.
CASE
A 36 year-old woman presented with one week of worsening pain in her back, lower extremities,
and nauseaand vomiting. She had a history of sickle cell anemia and a recent pulmonary
embolism Her vital signs were normal; she had hepatomegaly and tenderness in the right
upper quadrant. There was also pain in her back and lower extremities. Her alkaline
phosphatase was 71, total bilirubin 1.3, AST 1968, and ALT 2088. Her viral hepatitis
panel was negative; she denied alcohol use. Her acetaminophen and salicylate were
normal. She was treated for five days with intravenous fluids and pain medication,
and her liver enzymes decreased with resolution of her abdominal pain. She returned
ten days later; her has AST (53) and ALT (67) has both decreased.
DISCUSSION
Sickle cell disease is characterized by arterial occlusions due to micro-thrombi from
the sickled cells. Peripheral vaso-occlusion is the most common since systemic vascular
resistance is higher in these vessels and they are of smaller caliber. The result
is the typical bone and muscle pain of a sickle cell crisis. Solid organs can also
be involved, however, resulting in myocardial infarction, stroke, renal impairment
and, in this case, ischemic hepatitis. Although ischemic hepatitis is seen in only
ten percent of all sickle patients, physicians should be vigilant for the complication,
especially in the setting of right upper quadrant pain, hepatomegaly, jaundice, and
a low grade fever. The usual laboratory findings are elevated AST and ALT levels.
Treatment is supportive care with IVF and pain control; hepatitis that does not resolve
warrants exchange transfusions.
AN UNUSUAL CAUSE OF FEVER IN AN HIV+ PATIENT
R. Gardner
1. 1University of California, San Francisco, San Francisco, CA. (Tracking ID #117316)
LEARNING OBJECTIVES
1. Diagnose Multicentric Castleman's Disease. 2. Recognize the association between
Castleman's Disease, HHV-8, and HIV.
CASE
The patient is a 39 year-old HIV+ man (CD4 23) who presents with worsening fevers
and increasing abdominal pain for 1 week. He reports onset of intermittent fevers
18 months ago; 1 week ago, the fevers began to occur daily, accompanied by abdominal
pain, headaches, back pain, and nausea. The patient recalled a similar symptom constellation
7 months before that resolved spontaneously. He has Karposi's Sarcoma (KS) and takes
abacavir/3TC/AZT, lopinavir, tenofovir, azithromycin, and TMP/SMX. On admission the
patient was afebrile with normal vital signs. His exam was remarkable for axillary
and inguinal lymphadenopathy (LAD); a soft abdomen, mildly tender to palpation diffusely;
KS lesions on the left lower extremity. Laboratory studies showed anemia but were
otherwise normal. While hospitalized the patient spiked daily fevers, unrelated to
antiretroviral administration. Blood, urine, CSF and MAC cultures all were negative,
as were an influenza panel, monospot test, and cryptococcus serology. Chest radiography
and head CT were normal. Abdominal CT revealed splenomegaly and diffuse LAD. Biopsy
of an axillary lymph node showed Castleman's disease (CD) and stained positive for
human herpes virus 8 (HHV-8).
DISCUSSION
The differential diagnosis for fever and LAD, already extensive, is even broader in
patients with advanced HIV. In this case, the leading diagnoses included disseminated
TB, MAC, and non-Hodgkin's lymphoma. CD, which also manifests with fever and LAD,
is a rare lymphoproliferative disorder which has received renewed interest as increasing
case reports link it to HIV and HHV-8. The unicentric form of CD is isolated, usually
asymptomatic, and often discovered incidentally. Not typically associated with HHV-8,
it can be cured with surgical resection. Multicentric CD (MCD), as seen in this patient,
has systemic symptoms. Effective therapeutic options are limited, contributing to
a poor prognosis. The disease manifests differently in patients with and without HIV
and HHV-8. HIV+ patients with MCD are universally positive for HHV-8, and are more
likely to have a rapidly progressive course with a shorter survival. New studies suggest
that HHV-8 may contribute more to this pattern than HIV. Patients typically die of
fulminant infection or associated malignancies. Optimal treatment is unclear given
the rarity of the disease, the variety of clinical presentation, and the paucity of
literature. Most therapies offer a temporary response with relapse after discontinuation,
but combination chemotherapy and rituximab show promise for more durable responses.
Castleman's disease is a rare disorder with a clinical course shaped by the presence
of HIV and HHV-8; it should considered in any HIV+ patient with KS, fevers, and lymphadenopathy.
AN UNUSUAL CAUSE OF RECURRENT RHABDOMYOLYSIS
D.L. Stern
1; R. Warrier2; J. Fixley2; E. Adickes2; J. Derby3. 1Creighton University Medical
Center, Omaha, NE; 2CUMC, Omaha, NE; 3Creighton University, Omaha, NE. (Tracking ID
#115653)
LEARNING OBJECTIVES
1. Recognize that viral infections are a common cause of rhabdomyolysis. 2. Recognize
that a muscle biopsy is the gold standard for diagnosis of rhabdomyolysis. 3. Realize
that congenital diseases can have initial presentation in adulthood.
CASE
Case Presentation: A 23 yo AA girl with PMH of asthma was admitted to the hospital
with a 3 day history of generalized muscle pain. She reported upper respiratory tract
symptoms approximately one week prior to admission. She denied muscle weakness, changes
in urine color or urine output. She had no history of trauma, no new meds, seizures,
or extraordinary physical exertion. She had been admitted two other times with similar
symptoms in the past four years. These episodes were treated as rhabdomyolysis believed
to be precipitated by viral illnesses. Physical exam was unremarkable except she displayed
generalized muscle tenderness without any objective muscle weakness. Her CPK was 35,136
with a serum myoglobin 3207. Other labs of significance included potassium of 3.9,
BUN/Cr of 13.0/1.0. Phosphorus was 4.1, AST was 133, ALT was 64, while the remainder
of her liver function tests were normal. Complete blood count was normal. A urinalysis
showed a myoglobin of 32,300. Free carnitine, acyl carnitine, total carnitine, pyruvate
and lactate were all within normal limits. A diagnostic muscle biopsy was performed
which revealed Nemaline myopathy. Patient was treated for rhabdomyolysis and was asymptomatic
with low levels of CPK at the time of discharge.
DISCUSSION
Discussion: Nemaline myopathy is a congenital muscle disease with a wide spectrum
of phenotypes, ranging from forms with neonatal onset and fatal outcome to asymptomatic
forms. Muscle biopsy reveals atrophy, variation in muscle fiber size and a lattice
like appearance typical of nemaline rod bodies emanated from the Z-discs of affected
muscle fibers. Adult-onset cases usually manifest with symptoms as a child. Our patient
denied any problems as a child and was actually was very active in athletics. Most
case reports of adult-onset cases are of patients with progressive proximal weakness
or generalized weakness. These patients had either a normal or slightly elevated CPK.
None of these case reports displayed such a markedly elevated CPK or recurrent rhabdomyolysis
as manifested in our patient. We believe that this is the first case report of nemaline
myopathy presenting as recurrent rhabdomyolysis.
AN UNUSUAL ETIOLOGY OF LEFT INGUINAL LYMPHADENOPATHY IN A 53 YEAR OLD MAN
D. Takahashi
1; M.M. Schapira1; S.R. Pandit1. 1Medical College of Wisconsin, Milwaukee, WI. (Tracking
ID #116307)
LEARNING OBJECTIVES
1. Recognize unusual etiologies of localized lymphadenopathy. 2. Recognize clinical
features of Castleman's disease. 3. Determine when there is a need for biopsy in patients
presenting with lymphadenopathy.
CASE
A 53 year old Caucasian male presents with left groin lump and 10 kg weight loss over
a period of one month. The lump is painful to touch and has been progressively enlarging
in size over the past month. The patient also complains of generalized malaise and
night sweats. His past medical history is significant for essential hypertension.
The physical exam reveals an ill-appearing, 72 inch, 81 kg male with stable vital
signs. The general physical exam is unremarkable except for a left sided groin mass
measuring 2 –3 cm in size. The mass is discrete, firm and mobile with moderate tenderness
to touch and not associated with erythema or induration. Laboratory exam initially
reveals a normal CBC and differential and normal electrolytes. An ultrasound of the
groin mass reveals clusters of enlarged inguinal lymph nodes, the largest of which
measures 3.6 cm. A CT scan of the chest, abdomen, and pelvis reveals no additional
lymphadenopathy. An inguinal lymph node biopsy was done, with initial pathology impression
being a low grade lymphoma, but a second opinion from a reference laboratory was reported
as follicular hyperplasia with expanded mantle zones, atretic germinal centers and
monotypic lambda expression by plasma cells (Castleman's disease like changes).
DISCUSSION
Lymphadenopathy is often a diagnostic challenge for general internists. There is a
rather large list of possible etiologies, some of which require immediate attention
and management. Definitive diagnosis is often obtained by biopsy, which is invasive
and not necessary in some cases. The role of general internists is crucial to identifying
patients who require lymph node biopsy through a detailed history and physical exam.
Age, location, duration, and associated signs and symptoms aid in deciding when a
biopsy is necessary. The presence of enlarging lymphadenopathy and systemic symptoms
in this patient indicate the need for a lymph node biopsy. Castleman's disease or
angiofollicular lymph node hyperplasia is an uncommon etiology of lymphadenopathy
that was first described by Benjamin Castleman. Castleman's disease can present as
localized lymphadenopathy (unicentric Castleman's disease) or generalized lymphadenopathy
(multicentric Castleman's disease). These two forms of disease carry different prognoses.
Unicentric Castleman's disease, as was found in this case, is potentially curable
with surgical excision of lymph node. Multicentric Castleman's disease, in contrast,
has a median survival of only 8 to 14 months. Clinically, it is difficult to differentiate
Castleman's disease from other more malignant lymphoproliferative disorders. Most
cases of unicentric Castleman's disease are asymptomatic, the median age of the patient
is approximately 35, it occurs equally in males and females, and the median size of
the lesion is 5 to 9 cm. Unicentric Castleman's disease is of two subtypes: hyaline-vascular
(90%) and the plasma cell type. The hyaline-vascular type is considered a reactive
chronic lymphoid hyperplasia. The plasma cell type is considered to have an inflammatory
pathogenesis, either through chronic antigenic stimulation (i.e. infection) or via
an autoimmune mechanism. A plasma cell dyscrasia which includes polyneuropathy, organomegaly,
endocrinopathy, monoclonal gammopathy and skin changes (POEMS syndrome) is sometimes
associated with Castleman's disease. Interleukin-6 has been defined to have a role
in the pathophysiology of this disease, and the systemic manifestations of Castleman's
disease. In contrast, multicentric Castleman's disease usually occurs in patients
with a median age between 52 and 65, and presents with systemic symptoms such as fever,
malaise, weight loss, and peripheral lymphadenopathy. Hepatomegaly and splenomegaly
are also common findings at presentation. Mediastinal and abdominal lymphadenopathy
is less common at presentation although approximately 50% will progress to involve
mediastinal or abdominal lymph nodes.
ANAPHYLACTIC REACTION TO TOPICAL LIDOCAINE
A. Pleister
1; J.L. Sebastian1; M. Glisczinski2. 1Medical College of Wisconsin, Milwaukee, WI;
2Clement J. Zablocki VA Hospital, Milwaukee, WI. (Tracking ID #116735)
LEARNING OBJECTIVES
To recognize the potential for topical anesthetic agents to cause severe allergic
reactions, including anaphylaxis, in susceptible individuals.
CASE
On the day of admission, a 73-year old man underwent a surveillance cystoscopy for
transitional cell carcinoma of the bladder and dilation of a recurrent urethral stricture.
During an uneventful 30-minute outpatient procedure, the patient received no medications
other than 20 cc of a 2% lidocaine gel that was used as a topical anesthetic. Within
30 minutes of leaving the cystocopy suite, the patient developed intense pruritis
and a feeling that he was about to pass out. The patient's wife noticed that he was
markedly weak and pale and she immediately brought him to the Emergency Room. Initial
physical exam revealed a diaphoretic man with a blood pressure (BP) of 61/34 and a
weak pulse at 76 beats/minute. The chest and heart exams were normal but a diffuse
urticarial skin rash was noted on the trunk and extremities. The patient's BP responded
to the administration of two liters of IV normal saline and 50 mg of IV diphenhydramine.
Additional medical history revealed that the patient had a previous allergic reaction
to Bactrim that caused a similar urticarial skin rash. His active prescription list
included propylthiouracil, lisinopril, felodipine and simvastatin for treatment of
hyperthyroidism, hypertension and hyperlipidemia. None of these medications were new
and the patient specifically denied use of any over-the-counter medications, supplements
or nutraceuticals. A literature search determined that the formulation of topical
lidocaine used in this case contained sodium metabisulfite, a sulfite responsible
for causing allergic type reactions, including anaphylaxis, in susceptible individuals.
The patient's chart was subsequently marked ALLERGIC TO TOPICAL LIDOCAINE and an adverse
drug reaction form was filed with the hospital pharmacy. After a brief period of hospital
observation, the patient was discharged home in stable condition and an outpatient
allergy appointment was scheduled for further evaluation and sensitivity testing.
DISCUSSION
Although usually considered a benign agent, some commercially available formulations
of topical lidocaine contain additives that have the potential to cause serious allergic
reactions in susceptible individuals. This vignette reinforces the importance of obtaining
a thorough allergic history before beginning any new medication or using a new formulation
of a previously administered medication. Systemic absorption of topically applied
medications can cause serious, and potentially life-threatening, complications.
ANEMIA DUE TO LAMOTRIGINE
N. Milojkovic
1; M. Elnicki1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116715)
LEARNING OBJECTIVES
Learning Objectives: 1. Recognize possible causes of anemia in a young woman. 2. Recognize
the hematologic side effects of Lamotrigine.
CASE
A 28 yo African American female with a history of seizures was admitted for worsening
shortness of breath and weakness. Review of systems was otherwise negative. Her seizures
were partial complex seizures controlled with Lamotrigine and Levetiracetam. She had
been seizure free for over 2 years. Her family history was positive for thyroid disease
and iron deficiency anemia. She does not use alcohol, tobacco or street drugs. The
patient's vital signs were normal except for a regular heart rate of 108, and her
physical exam was otherwise completely normal. Laboratory evaluation revealed Hemoglobin
of 4 mg/dL, Hematocrit 8.8%, MCV 93 fL, MCH 32 pg, MCHC 34 g/dL, Platelets 117.000/mcL,
WBC 3.000/mcL with 45% neutrophils, 2% bands, 42% lymphocytes, 3% monocytes, 8% nucleated
RBCs, reticulocyte count 5%. Serum iron was 150 µg/dL, transferrin saturation 60%
and ferritin 600 ng/mL. LDH, haptoglobin and indirect bilirubin were normal as well
as her TSH, B12, folate, LFTs and sickle cell prep. Viral antibody titers were negative.
Bone marrow biopsy showed trilineage hematopoiesis, marked erythroid predominance
and marked megaloblastic changes.
DISCUSSION
In this patient with anemia and seizure disorder, the differential diagnosis of severe
anemia includes: 1. Hypoproliferative bone marrow (e.g. medication induced, viral
infections, leukemic infiltration) 2. Maturation disorders including thalassemia,
sideroblastic anemia; B12 or folate deficiency. 3. Hemorrhage or hemolysis. We can
narrow our differential diagnosis based on the initial negative evaluation. The patient's
bone marrow has a poor reticulocyte response to this degree of anemia suggesting inefficient
erythropoiesis. Viral infections affecting the bone marrow, particularly Parvo B19,
can cause an aplastic crisis. However, her parvovirus antibodies were negative. The
bone marrow biopsy with evidence of megaloblastic picture in the setting of normal
B12, folate can be attributed to medications, particularly to Lamotrigine. Erythroblastopenic
crisis secondary to Lamotrigine has been described. Inhibition of dihydrofolate reductase
is probably the mechanism of erythroblastopenia and it occurs more often in patients
with underlying abnormal hematopoiesis, such as heterozygous [beta]-thalassemia. That
brings the question of possible underlying abnormality of hematopoiesis in our patient
and need for further work up such as hemoglobin electrophoresis. Treatment with folinic
acid results in complete resolution of the erythroblastopenic crisis. Using this therapeutic
approach, long-term treatment with Lamotrigine can be administered without any further
complication. However, because of the severity of anemia in our patient Lamotrigine
was discontinued and her hemoglobin recovered.
AORTODUODENAL FISTULA – A DIAGNOSTIC CHALLENGE
N. Nathan
1; K. Muniyappa1; S. Parikh1; H. Friedman1. 1St. Francis Hospital, Evanston, IL. (Tracking
ID #116043)
LEARNING OBJECTIVES
1. A high index of suspicion for ADF based on history and physical examination should
be maintained in the presence of equivocal or even negative diagnostic tests. 2. The
finding of a primary aortoduodenal fistula in a patient with previous aortic aneurysm
repair is extremely rare.
CASE
An 84-year-old male with past medical history of hypertension, congestive heart failure,
aortic valve replacement (on warfarin) and AAA repair, presented with one episode
of bright red blood per rectum. On physical examination, he was afebrile and vitals
were stable. Orthostatic hypotension was absent. Hemoglobin was 12 gm/dL, BUN was
55 mg/dL, serum creatinine was 1.8 mg/dL and INR was 2.99. An emergent EGD revealed
no active bleeding. An abdominal CT scan with IV contrast revealed a saccular aneurysm
arising from the infrarenal abdominal aorta with contrast entering the distal duodenum.
This was highly suspicious for an aortoenteric fistula. Vascular surgery was immediately
consulted. The patient suffered another episode of bright red blood per rectum with
massive exsanguination and died despite aggressive resuscitation efforts. An autopsy
evidenced a fistulous communication between the atherosclerotic aneurysm of orta and
the third part of the duodenum, proximal to the suture line of the previous vascular
graft. This was consistent with a primary aortoduodenal fistula, as defined below,
and was quite unexpected.the a
DISCUSSION
A primary aortoduodenal fistula (PADF) is defined as a communication between the native
aorta and the duodenum. In contrast, secondary aortoduodenal fistulas (SADF) arise
between the suture line of a vascular graft and the duodenum and are far more common
than PADFs. The most frequent cause of PADF is atherosclerosis. The triad of pain,
GI bleeding and an abdominal mass is seen in only 40% of patients. The initial bleeding,
commonly known as a `herald bleed', is often transient and self limiting owing to
thrombus formation. EGD is useful to refute other causes of GI bleeding, but does
not rule out ADF, which is often in the distal duodenum. Abdominal CT is specific
but has low sensitivity for ADF. Treatment consists of emergent exploratory laparotomy
with graft repair of the aorta and closure of the fistula tract. A high index of clinical
suspicion, based on history and physical examination, is the key to correct diagnosis.
Although the suspicion for SADF was high, the presence of a PADF in our patient makes
this case unique.
APLASTIC ANEMIA IN PREGNANCY
V.R. Patel
1; N. Le1. 1Baylor College of Medicine, Houston, TX. (Tracking ID #116919)
LEARNING OBJECTIVES
1) Review the presentation of aplastic anemia in pregnancy 2) Review the possible
pathogenesis and current treatment options of aplastic anemia in pregnancy.
CASE
A 26 year old G2P1 16 week pregnant African American woman with a history of sickle
cell trait and iron deficiency anemia was referred by her doctor for an abnormal CBC:
WBC 4.1 (62N, 2B, 31L, 5M), Hgb. 9.1 (MCV 98.5) and platelets of 11K. The patient
reported fatigue for several months and easy bruising for 2 years. The patient had
a prior uncomplicated pregnancy with a vaginal delivery on 10/01 except for iron deficiency
anemia and thrombocytopenia (70K). The patient's vital signs included a temperature
of 98.1F, BP 109/64, and HR 88. On exam there was a 2 cm. ecchymosis on her left thigh
and numerous petechiae on her chest. Pertinent laboratory results included normal
PT, PTT, folate, B12, TSH. Her reticulocyte index was .6, Ferritin 14, and Iron Sat.
31%. Bone marrow biopsy revealed markedly hypocellular marrow (20%) with severely
decreased megakaryocyte and myeloid cell content and no tumors or other abnormalities.
She was treated successfully with multiple platelet and blood transfusions, along
with cyclosporine, antithymocyte globulin (ATG), and corticosteroids.
DISCUSSION
Aplastic anemia (AA) in pregnancy is rare, but has long been recognized. Currently,
there is no proven association between pregnancy and AA. Fleming postulated a hormonal
mechanism due to an imbalance between erythropoietin and placental lactogen which
both increase erythropoiesis and estrogens which inhibit hematopoiesis. This idea
could be supported by the spontaneous resolution of aplasia in 1/3 of cases after
delivery. Historically, AA results in a high maternal and fetal morbidity and mortality.
However, in recent years with modern supportive therapy, maternal mortality has been
15% and more than 90% of patients survive in remission. Treatment options depend on
the timing of AA during pregnancy and its severity. AA occurring early in pregnancy
may be treated by termination of pregnancy and if necessary a bone marrow transplant.
AA occurring in the 2nd half of pregnancy should be treated with ATG +/– cyclosporine
and supportive care. ATG is effective for those with non-severe AA who are transfusion
dependent and for severe AA in the absence of an HLA compatible sibling. ATG used
in combination with cyclosporine results in a 50% survival rate. Supportive care remains
the main treatment and includes the minimization of transfusions to decrease the likelihood
of sensitization. Transfusion with HLA-matched or single-donor platelets is recommended.
ARTERIAL EMBOLUS PRESENTING AS ACUTE ABDOMINAL PAIN
M. Roschewski
1. 1Eisenhower Army Medical Center, Augusta, GA. (Tracking ID #116713)
LEARNING OBJECTIVES
1. Recognize challenges of making diagnosis of arterial embolus 2. Identify risk factors
for arterial emboli 3. Recognize management difficulty in patients with unidentifiable
cause of arterial emboli.
CASE
30 y.o. African-American female without significant medical history presented to the
emergency department after experiencing the acute onset of sharp, right-sided abdominal
pain. Contrasted abdominal CT revealed the presence of a wedge-shaped infarct in the
right renal cortex consistent with infarcted tissue. Magnetic resonance imaging of
the kidney and its associated arteries confirmed the diagnosis of arterial embolism.
Transesophageal echocardiogram and renal arteriogram was unable to identify a source
of embolus. Extensive laboratory evaluation to identify an inherited or acquired thrombophilia
was negative. Despite the uncertain etiology of the renal infarction, our patient
was placed on warfarin anticoagulation indefinitely. Six months later our patient
presented again with acute onset of chest pain and EKG changes of her inferior leads.
Her cardiac troponin enzymes were elevated, revealing ongoing myocardial infarction.
Coronary arteriography revealed the presence of a thrombus in her right coronary artery
without associated atherosclerotic disease.
DISCUSSION
Systemic arterial emboli have variable presentations that range from asymptomatic
to the acute onset of abdominal pain mimicking surgical emergencies. Arterial embolic
phenomena, in general, most often affect older persons with mural thrombus from atrial
fibrillation or erosive atheromatous disease following aortic manipulation. Such phenomena
have, however, also been described as the presenting feature of inherited and acquired
thrombophilias such as antiphospholipid antibody syndrome, hyperhomocysteinemia, and
paroxysmal nocturnal hemoglobinuria. This case illustrates the challenge in making
diagnoses as well as long-term management in patients who present with unusual embolic
phenomena of arterial origin. Despite advances in our understanding of thrombophilias,
up to 20% of patients will remain undiagnosed and be subjected to years of anticoagulation
without well-studied outcomes.
AUTOIMMUNE HEPATITIS MASQUERADING AS HEMOCHROMATOSIS
B. Konicek
1; J. Franco1; J.L. Sebastian1; D. Torre1. 1Medical College of Wisconsin, Milwaukee,
WI. (Tracking ID #116751)
LEARNING OBJECTIVES
1) Recognize the clinical presentation and differential diagnosis of autoimmune hepatitis
(AIH) and 2) recognize iron overload as a condition that can accompany many types
of acute liver injury.
CASE
A 57-year-old Caucasian man with a history of type 2 diabetes and alcohol use of 40
g/day presented with a one-week history of anorexia, fatigue and dark-colored urine.
He denied any history of fever, chills, abdominal pain, pruritis, hematuria, myalgias
or change in bowel habits. There was no past history of HIV disease, blood transfusions,
IV drug use, exposure to sick contacts or excessive use of acetaminophen. Because
the patient was adopted, no family history was available. Physical exam was remarkable
for normal vital signs, tanned skin, scleral icterus and hepatomegaly. Admitting lab
tests revealed a total bilirubin of 19.2 mg/dl, AST = 3093 U/L, ALT = 2092 U/L, alkaline
phosphatase = 171 IU/L, protime = 14.1 seconds, ferritin = 7497 ng/ml and ceruloplasmin
= 36 mg/dl. Viral hepatitis titers were all negative as were the titers for ANA and
anti-mitochondrial antibodies. Anti-smooth muscle antibody titers were mildly elevated
at 1 : 80. An abdominal CT scan demonstrated diffuse hepatosplenomegaly without dense
changes in the liver parenchyma or mineralized lymph nodes typical of hemochromatosis.
Liver biopsy revealed a minimal amount of stainable iron within hepatocytes and thin,
bridging fibrocollagenous strands between hepatocytes. After two weeks, aminotransferase
levels declined to 2–3 times normal, but the patient continued to complain of anorexia
and fatigue. Follow-up blood work three months later revealed a serum gamma globulin
level of 1.62 g/dl, elevated IgG = 1800 mg/dl and IgM = 501 mg/dl. Taken together,
all of these findings suggested a probable diagnosis of AIH.
DISCUSSION
Autoimmune hepatitis is a rare disorder of unknown etiology characterized by persistent
inflammation of the liver. Clinical manifestations of AIH display marked variability,
ranging from asymptomatic periods to fulminant hepatic failure. The diagnosis of AIH
is based on a scoring system that includes gender, drug/alcohol history, immunoglobulin
levels, antibody titers, viral hepatitis markers, liver histology, HLA serotypes and
the presence of additional autoimmune diseases. Although our patient was a middle-aged
male, AIH is usually more common in women (3.6 : 1) and younger patients (peak incidence
ages 16–30). This clinical vignette reminds clinicians that iron overload can accompany
any form of acute hepatitis and reinforces the need to include AIH in the differential
diagnosis of suspected hemochromatosis.
AUTOIMMUNE THROMBOCYTOPENIC PURPURA AND GRAVES' DISEASE: A CASE OF WORSENING THROMBOCYTOPENIA
DUE TO THYROTOXICOSIS
J. Baez-Escudero
1; F. Grzywacz1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116186)
LEARNING OBJECTIVES
1. Recognize the rare association between Autoimmune Thrombocytopenic Purpura and
Graves' disease. 2. Recognize that treatment of Graves' disease with return to an
euthyroid state can improve thrombocytopenia.
CASE
A 23 year old nulliparous Vietnamese woman with Idiopathic Thrombocytopenic Purpura
(ITP) presented with palpitations, hair loss, tremor, anxiety, weight loss, heat intolerance
and fatigue. She had been diagnosed with steroid responsive ITP two years prior. She
denied history of bleeding complications. She had stable platelet counts of 20,000
on 20 mg of oral prednisone. On exam, she was tachycardic. She had a tremor, exophthalmos,
mild epistaxis, gingival bleeding, diffuse nontender non-nodular goiter, hyperreflexia,
and a severe petechial rash. Platelet count was 8,000. Her thyroid studies were: TSH
0.00, Total T3,714.9 (80–220 ng/dl), Total T4 24.5 (4.5–12.5 mcg/dl), Free T4 5.6
(1.0–2.3 ng/dl). Thyrotropin receptor autoantibodies were positive. Thyroid radioactive
iodine uptake scan showed diffusely increased uptake. Serum beta-hcg and HIV were
negative. Graves' disease was diagnosed and propranolol was started for symptomatic
treatment. She was given a higher dose of prednisone for her worsening thrombocytopenia,
but she did not respond. Due to the risk of bleeding, the patient was not offered
a subtotal thyroidectomy. She was treated with a one time dose of radioactive iodine
(131 I) after which she became euthyroid. Four weeks after treatment she was tapered
off prednisone and maintained platelet counts above 50,000 for the next few months
without further need for steroid therapy.
DISCUSSION
Autoimmune Thrombocytopenic Purpura is a rare but previously reported hematologic
manisfestation of Graves' disease. The association between hyperthyroidism and thrombocytopenia
is a known although infrequent occurrence. Distinct mechanisms are probably active
in each condition, but the thyrotoxic state has been implicated as having a key effect
on the fall in the number of platelets. Theories for this event include a common immunologic
cause or a thyrotoxic-induced decrease in platelet survival. We describe a patient
with coexisting Graves' disease and “idiopathic” thrombocytopenic purpura who showed
minimal response to treatment of thrombocytopenia in the thyrotoxic state, but who
promptly recovered and was able to sustain higher platelet counts while she was euthyroid.
Evaluation of the thyroid condition in patients with refractory thrombocytopenia is
advised. Specific therapy for the hyperthyroid state might lead to a moderate increase
of the platelet count.
B12 DEFICIENCY AND DRUG ABUSE: N0 LAUGHING MATTER
M.T. Reyes
1; M. Rotblatt2. 1UCLA-SFVP Olive View Medical Center Department of Internal Medicine,
Sylmar, CA; 2UCLA SFVP-Olive View Medical Center Department of Internal Medicine,
Sylmar, CA. (Tracking ID #115053)
LEARNING OBJECTIVES
1. Recognize nitrous oxide (N2O) exposure as a cause of subacute combined degenerative
(SCD) myelopathy. 2. Recognize the neurologic deficits associated with a dorsal column
myelopathy.
CASE
A 24 year old Latino male presented with 2 weeks of progressively worsening balance
problems and numbness in his distal extremities. His symptoms started as a tingling
sensation in the fingers of both hands and both feet, later progressing to clumsiness
with fine motor movements of his hands. Mild balance problems progressed to being
chair-bound at the time of admission. He denied taking any medications, but admitted
to binge drinking alcohol roughly twice a month for 2 years. He reported having a
conventional Western diet. Physical examination was significant for diminished vibration
sense and proprioception in both hands and feet, but preserved sensation to light
touch and pinprick. His cranial nerves and DTR's were normal. The only weakness noted
was in the intrinsic muscles of his hands. Marked ataxia, with a wide-based and unsteady
gait, and mild finger-to-nose and heel-to-shin dysmetria, were present. After the
initial assessment, the patient's wife disclosed that he had been abusing inhaled
N2O for 5 years, increasing his use in the previous 4 weeks. Basic labs were normal.
MRI of the C-spine showed increased T2 signal of the posterior columns bilaterally,
suggestive of B12 deficiency. Serum B12 level was 110 pcg/ml (normal 250–1000), and
methylmalonic acid level was 7236 (90–279). He was started on B12 injections and was
transferred to a rehabilitation facility. After 2 weeks of B12 injections and PT,
the patient showed mild improvement in his ataxia and sensory complaints. He was subsequently
lost to follow up.
DISCUSSION
Layzer et al. first described neuropathy after recreational nitrous oxide (laughing
gas) abuse in 1978. Subsequently there have been about 10 published cases of a similar
presentation either after exposure to N2O anesthesia or chronic recreational use.
The symptoms are consistent with findings of B12 deficiency, that is, the classic
picture of subacute combined degeneration of the dorsal and lateral spinal columns.
N2O oxidizes cobalamin into its inactive form, thereby causing B12 deficiency. The
methionine synthase enzyme and myelin production are inhibited, thus producing the
classic neurologic manifestations. Clinically, patients present with parasthesias
and ataxia, with diminished vibration sense and proprioception. Patients may or may
not have macrocytic anemia. MRI findings typically reflect the pathophysiology of
dorsal column demyelination of the cervical spinal cord. Symptoms and MRI findings
may be reversible if B12 replacement is initiated early.
BACILLARY PELIOSIS HEPATITIS: IF SCRATCH DEEP ENOUGH, YOU'LL GET THE DIAGNOSIS
S. Irani
1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116209)
LEARNING OBJECTIVES
1. Review the diseases caused by Bartonella species. 2. Recognize bacillary peliosis
as an important differential in HIV patients with fever and abdominal pain. 3. Review
diagnosis and treatment strategies for peliosis hepatitis and splenitis.
CASE
A 34 year old African American female with AIDS (last CD4 207) presented with two
week history of fever, malaise, nausea, vomiting, diarrhea, diffuse abdominal pain.
On exam, she had T = 102.7, small 1cm sub-mandibular nodule, soft diffusely tender
abdomen. Lab data revealed WBC 12.7 without shift, Hgb 7.5. Blood chemistries, liver
function tests, blood cultures, stool studies, CXR were all negative. CT abdomen showed
multiple 2–7 mm lesions in liver and spleen. During admission, her diarrhea resolved,
but fever and malaise persisted. Upon further questioning, patient recalled being
scratched one month prior by stray cats that she fed to a ward of rats. Liver biopsy
revealed scattered granulomatoid inflammation with rod shaped bacilli on Warthin Starry
stain. Electron micrograph showed multiple trilaminar cell-walled bacillary organisms
leading to a diagnosis of bacillary peliosis hepatitis.
DISCUSSION
The genus Bartonella contains four species demonstrated to be pathogenic in humans
(bacilliformis, henselae, quintana, elizabethae). Disease syndromes are of variable
severity, ranging from cat-scratch disease (CSD) to systemic diseases such as trench
fever and bartonellosis. In general, immunocompetent patients who are otherwise healthy
tend to present with classic CSD. Patients who are immunocompromised tend to have
systemic manifestations such as bacillary angiomatosis, extracutaneous lesions, bacteremia,
and bacillary peliosis hepatitis and splenitis. Bacillary peliosis was first reported
in HIV patients in 1990. It is the occurrence of multiple blood-filled cysts in the
liver and/or spleen. Biopsy remains the gold standard for diagnosis, open having higher
yield than percutaneous or transvenous. First line treatment is with erythromycin
and doxycycline. Optimal duration of treatment is unknown, but most sources recommend
3 months. Relapses are not uncommon and usually occur with shorter courses of treatment.
Our case highlights the importance of including bacillary peliosis in the differential
of fever and abdominal pain in HIV patients.
BACTEREMIA IN A NURSING HOME RESIDENT: LOOK BEYOND THE FOLEY
S. Kumar
1; E. Anish1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115905)
LEARNING OBJECTIVES
1. To recognize diverticulitis as a potential source of Providencia stuartii bacteremia.
2. To appreciate an atypical presentation of diverticultis.
CASE
A 77 year-old female, nursing home resident presented with a one-week history of malaise
and chills. Her PMH was significant for CAD, HTN, and urinary incontinence that resulted
in the placement of a chronic, indwelling Foley catheter. Physical exam revealed an
elderly appearing female in no acute distress. She was afebrile with a BP-126/78 mmHg
and HR-78 beats/minute.The remainder of her exam was unremarkable with no localizing
signs of infection. Initial diagnostic tests revealed a UA significant for 5–10 WBCs.
CBC and serum chemistries were WNL. A CXR was unremarkable. After obtaining blood
and urine cultures, empiric antibiotic therapy with levofloxacin was initiated for
a presumptive diagnosis of a urinary tract infection. The next day, 2 sets of blood
cultures returned positive for Providencia stuartii, resistant to levofloxacin. The
urine culture revealed no growth. The patient's antibiotics were changed to piperacillin/tazobactam
and gentamicin and a work-up was initiated to determine the source of the bacteremia.
Additional testing included an ultrasound of the abdomen/pelvis and a transthoracic
ECHO that were both unremarkable. A CT scan of the abdomen/pelvis revealed mild sigmoid
diverticulosis, but no other abnormalities. An indium-labeled WBC scan was then performed
that showed increased activity in the right lower quadrant of the abdomen. Based on
this result, her prior abdominal CT was re-evaluated and, in fact, changes consistent
with cecal diverticulitis were found. A 14-day course of IV antibiotics was completed
and the patient's symptoms resolved. Of note, throughout the entire illness, the patient
remained asymptomatic from a GI standpoint and she never demonstrated any abdominal
tenderness on exam.
DISCUSSION
Most cases of Providencia stuartii bacteremia occur in patients who develop catheter-related
urinary tract infections. Although Providencia stuartii can be found as a component
of the normal bowel flora, it is not a common organism associated with bacteremia
in the setting of gastrointestinal disease. In fact, a MEDLINE search revealed no
published cases of Providencia stuartii bacteremia related to diverticulitis. Although
our patient had a Foley catheter, the absence of bacteriuria compelled a search for
an alternative source of her bacteremia. This patient lacked the more common manifestations
of diverticulitis (i.e., fever, abdominal pain and tenderness, leukocytosis), but
a diagnosis was able to be made based on the results of diagnostic imaging studies.
This case serves to illustrate how diverticulitis can present in an unusual manner
in an older adult and it emphasizes the importance of re-evaluating prior diagnostic
tests when necessary, even if the initial reports are unremarkable.
BE SENSITIVE TO SENSITIVITY: ASPIRIN-INDUCED ASTHMA
C.L. Spagnoletti
1; M.A. McNeil1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115007)
LEARNING OBJECTIVES
1) Recognize the clinical presentation of aspirin-induced asthma (AIA) 2) Understand
the pathophysiology of AIA 3) Discuss the treatment of AIA.
CASE
Mr. A is a 68 year old male who was hospitalized with back pain secondary to a compression
fracture. His past medical history was significant for asthma, nasal polyps, and osteoporosis.
His medications included fluticasone plus salmeterol inhaler and alendronate. He reported
an allergy to aspirin, but was unable to provide further details. He denied alcohol
or tobacco use. On exam, he was afebrile with stable vital signs. His oxygen saturation
was 98% on room air. His pulmonary exam revealed no wheezes or rhonchi and good air
movement. He was given intravenous etodolac for pain relief. One hour later, he developed
severe shortness of breath and nasal congestion, with a drop in his oxygen saturation
to 80% on room air. His repeat pulmonary exam revealed decreased air movement and
diffuse inspiratory and expiratory wheezes. The CXR was clear. He was felt to have
developed bronchospasm secondary to etodolac and was treated with albuterol/atrovent
nebulizers, oxygen, and intravenous steroids. His symptoms resolved within 12 hours.
DISCUSSION
Five to fifteen percent of asthmatics are intolerant to aspirin, and aspirin intolerance
(AI) is a risk factor for the development of asthma. AI is more common in asthmatics
with nasal polyps and sinusitis. Most asthmatics who are sensitive to aspirin are
also sensitive to non-steroidal anti-inflammatory medications (NSAIDs). AIA usually
presents in the third or fouth decade in individuals who were not previously sensitive
to aspirin or NSAIDs. Patients with AI tend to have more severe asthma than those
without. Wheezing, congestion, rhinorrhea, tearing, facial flushing, or angioedema
occur one-half to two hours after ingestion. The pathogenesis relates to the metabolism
of arachnidonic acid. Patients with AIA react to compounds that inhibit cyclooxygenase
(COX) I, which catalyzes the formation of prostaglandins and thromboxanes from cell
membrane arachnidonic acid. These inhibitors include aspirin and most NSAIDs. When
COX I is inhibited, arachidonic acid metabolism is shunted to the 5-lipoxygenase pathway,
the byproducts of which are leukotrienes. They are potent inflammatory mediators which
can induce bronchconstriction, mucus secretion, nasal mucosal swelling, airway edema,
and attract eosinophils into the airways in predisposed people. AIA patients have
increased amounts of 5-lipoxygenase pathway enzymes. Non-AIA asthmatics do not overproduce
leukotrienes in response to COX I blockade. Treatment of AIA is according to usual
asthma guidelines. Leukotriene modifying drugs are pivotal in long term management.
Aspirin and NSAIDs should be avoided. Desensitization can be done if either is absolutely
necessary. COX 2 inhibitors are safe for use.
BILATERAL CORTICAL BLINDNESS AS PRESENTING SYMPTOM OF INFECTIVE ENDOCARDITIS (IE)
P. Cunningham
1; P. Radhakrishnan2. 1St. Joseph Hospital, Phoenix, AZ; 2St Joseph Hospital, Phoenix,
AZ. (Tracking ID #116483)
LEARNING OBJECTIVES
LEARNING OBJECTIVES: 1. Diagnose cortical blindness. 2. Recognize the rare association
of IE and occipital infarction.
CASE
A 41 year old female with a history of untreated hypertension and IV drug use presented
with history of sudden onset of blurring of vision, stating that she was unable to
distinguish any objects. She also complained of weakness on her left side and mild
shortness of breath. PHYSICAL EXAM: The patient was obese . There was mild confusion.
Vital signs were normal except for a temperature of 39.0 C. Eye exam revealed normal
fundi, PERL, no light perception. There were track marks on the arms, bilateral splinter
hemorrhages, and Janeway lesions. There were mild sensory and motor deficits in the
upper extremities. LABS: MRI brain: bilateral occipital and subcortical infarcts c/w
septic emboli. CT Chest: multiple airspace densities bilaterally c/w septic emboli.
WBC 11.6 with normal differential, ESR 39, Drug screen +cocaine. HOSPITAL COURSE:
Patient was treated with antibiotics for presumptive endocarditis with significant
sequelae of CVA w/residual left-sided partial paralysis, blindness and pulmonary emboli.
Transesophageal echocardiogram (TEE) showed a large tricuspid vegetation, moderate
mitral vegetation, and a patent foramen ovale (PFO) by bubble study. Blood and sputum
cultures: Oxacillin Sensitive Staphylococcus aureus. There was no evidence of aneurysm
per CT angiogram. The patient experienced a slight improvement in vision prior to
hospital discharge with return of light perception.
DISCUSSION
IV drug use accounts for half of IE cases in developed countries. The tricuspid valve
is involved in 30–70% of IVDU-related IE, Staphylococcus aureus accounting for 50%
of IVDU IE. PFOs are estimated to be present in 30% of the normal population. While
embolization is a common complication of IE, the incidence of neurologic complications
being 20–40%, cortical blindness is exceedingly rare. Cortical blindness is defined
as bilateral visual defects with normal ocular and pupillary exam, most often caused
by lesions of the vertebral-basilar arteries, often due to atherosclerosis, malformations.
Review of literature has revealed a handful of case reports of infective endocarditis
causing cortical blindness. The causes have ranged from rupture of a mycotic aneurysm,
infarction to TIA due to septic emboli. Our case has several interesting aspects.
Cortical blindness due to embolic stroke caused by infective endocarditis. The presence
of Patent Foramen Ovale in this patient was possibly responsible for the widespread
dissemination of the emboli. To summarize, consider infective endocarditis in the
differential diagnosis of cortical blindness, in a young patient.
BREAST MASS AS AN ATYPICAL PRESENTATION OF BLASTOMYCOSIS
B.A. Kisiel1; A. Marhatta
1; K. Rajkotia1; H. Friedman1. 1St. Francis Hospital, Evanston, IL. (Tracking ID #116244)
LEARNING OBJECTIVES
Distinguish breast involvement of Blastomycosis from breast malignancy.
CASE
A 48-year-old woman presented with dry cough, generalized weakness and a right breast
mass for one month. She denied fever, chest pain or hemoptysis. She was not a smoker
and denied alcohol use. Physical exam: afebrile; BP: 135/80 mmHg; pulse: 70/min; RR:
14/min. Breast exam: 3 by 2 cm nontender, erythematous nodule within the outer quadrant
of the right breast. Lungs: Diminished breath sounds at the right base. Remaining
exam was unremarkable. Laboratory data: WBC 28.000 (83% neutrophils). AFB sputum and
HIV test were negative. CT chest revealed right middle and lower lobes consolidation
with pretracheal lymphadenopathy and the right breast mass was identified. Mammogram
confirmed the mass in the right breast and showed a smaller mass within the left breast.
The patient underwent bronchoscopy and breast biopsy. Histopathological analysis of
transbronchial biopsy and breast tissue revealed broad-based budding yeast accompanied
by chronic granulomatous inflammation. A diagnosis of blastomycosis was made which
was further confirmed by positive fungal cultures. The patient was started on Itraconazole
with significant clinical improvement.
DISCUSSION
Blastomycosis is a systemic pyogranulomatous infection caused by the dimorphic fungus
Blastomyces Dermatitidis. Initial infection results from inhalation of the conidia
into the lungs. The clinical spectrum is varied, including asymptomatic infection,
acute or chronic pnemonia, and disseminated disease involving the skin, bones and
CNS. Alveolar infiltrates, mass lesions, and fibronodular infiltrates are the most
common radiographic findings. Disseminated disease in immunocompetent hosts is uncommon.
Blastomycosis of the breast is reported infrequently and clinically mimics carcinoma.
Treatment of choice for mild to moderate disease is Itraconazole for 6–12 months.
Amphotericin B is used for severe disease and CNS involvement. In patients presenting
with breast and pulmonary masses blastomycosis in addition to cancer should be considered.
(attached illustrations are the mamogram and the silver methenamine staining of the
breast tissue)
BRONCHIECTASIS IN A PREGNANT PATIENT
B. Springgate
1; M. Landry1. 1Tulane Health Sciences Center, New Orleans, LA. (Tracking ID #117516)
LEARNING OBJECTIVES
1. Recognize major diagnoses associated with bronchiectasis. 2. Identify occult cystic
fibrosis in the adult patient. 3. Demonstrate the need for optimization of health
status and genetic counseling for cystic fibrosis patients considering pregnancy.
CASE
A 32 year-old woman was transferred to the intensive care unit (ICU) with purulent
respiratory secretions following delivery of twins at 32 weeks gestational age. Her
follicle-stimulation-assisted pregnancy was complicated by mild cough, transient pruritus,
and moderate elevations of liver transaminases, alkaline phosphatase, and amylase
at 27 weeks gestation. In the ICU, the chest x-ray demonstrated diffuse patchy infiltrates.
Sputum culture revealed pan-sensitive Staphylococcus aureus. The patient rapidly improved
on intravenous antibiotics. An outpatient CT of the chest revealed diffuse bronchiectasis,
and a CT of the abdomen failed to demonstrate a gall bladder. Sweat chloride levels
were 102.5 and 91.0 meq/L, and pulmonary function testing revealed FEV1of 31% of the
predicted value.
DISCUSSION
Bronchiectasis is associated with allergic bronchopulmonary aspergillosis, hypogammaglobulinemia,
immotile cilia syndrome, and cystic fibrosis (CF). Cystic fibrosis is uncommonly diagnosed
during pregnancy. Clues to diagnosis may include evidence of infertility, mild sinus
or pulmonary disease, cholestasis, pancreatitis, and absent gallbladder. Genetic counseling
and family planning assume paramount importance in patients with CF, as prognosis
for mother and infant can be adversely affected in the setting of advanced lung or
pancreatic disease.
CAN CHEMOTHERAPY INDUCE RADIATION-LIKE EFFECT? RADIATION RECALL MYOSITIS INDUCED BY
GEMCITABINE
A.P. Amin
1. 1John H. Stroger Jr. Hospital of Cook County, Chicago, IL. (Tracking ID #116671)
LEARNING OBJECTIVES
1. To recognize a rare cause of leg swelling in a cancer patient. 2. Beware of the
radiation recall phenomenon. 3. To emphasize the association of 'radiation recall
phenomenon' with gemcitabine.
CASE
A 47-year-old male with pancreatic cancer, metastasized to the right hip and femur
bone, received a ten-day course of radiation (300 cGy daily) to the right hip and
thigh. On completion of radiotherapy, there were no skin changes or swelling of the
right thigh. One month later the patient was started on weekly gemcitabine infusions
(1000 mg/m2) as palliative chemotherapy — which he received for six weeks. On presentation
for the 7th week of chemotherapy, he complained of right thigh pain, swelling and
inability to walk. On examination, he was afebrile. The right and left thighs measured
32 cm and 18 cm respectively. The swelling was strictly confined to the area of previous
irradiation. A CT scan revealed diffuse, massive enlargement of all muscles in the
right thigh. There was no localized abscess or pus collection. The creatinine kinase
(CK) was elevated (624 U/L) and the total leukocyte count was normal (3,400/mm3).
A duplex scan for deep vein thrombosis was negative. A diagnosis of radiation recall
myositis was made on clinical grounds, and chemotherapy was stopped.
DISCUSSION
Radiation recall phenomenon is an acute inflammatory reaction that develops exclusively
at a previously irradiated site, in response to an anti-neoplastic agent. The anti-neoplastic
agent triggers the `recall' of the effect of radiation given earlier, and the quiescent
tissue becomes inflamed as if freshly irradiated. Recall reactions have been described
by various agents (doxorubicin, paclitaxel, etoposide, gemcitabine, bleomycin, vinblastine)
and at various sites (skin, muscle, lung, oral mucosa, vagina). Of all recall reactions,
dermatitis is the most common while myositis is quite rare. Only three cases of radiation
recall myositis have been previously reported, all induced by gemcitabine. The exact
mechanism of radiation recall reactions remains unclear. Possible hypotheses include
radio-sensitization by the precipitating agent, a drug hypersensitivity-like mechanism,
a Koebner phenomenon like reaction and radiation-induced up-regulation of thymidine
phosphorylase. No treatment guidelines currently exist for this condition. Generally
the triggering agent is discontinued, and there are anecdotal beneficial results with
steroids. Clinicians should be aware of this unusual complication of cancer treatment.
CARDIAC TAMPONADE: A RARE COMPLICATION OF ADULT ONSET STILL's DISEASE
L. Crawford
1; M. Panda1; R. Enzenauer1. 1University of Tennessee, Chattanooga, chattanooga, TN.
(Tracking ID #115170)
LEARNING OBJECTIVES
To recognize the clinical features of Adult Onset Still's Disease (AOSD) and the potentially
fatal complication of cardiac tamponade.
CASE
A 19 year old white female with a past history of Hodgkin's lymphoma currently in
remission and recently diagnosed AOSD presented with the complaints of chest pain
and shortness of breath. The chest pain was sharp, 8/10 in severity, exacerbated by
deep inspiration and coughing and it radiated posteriorly. This was associated with
severe dyspnea on exertion, paroxysmal nocturnal dyspnea and orthopnea. Leaning forward
relieved both symptoms. She described recent subjective fevers, diaphoresis, diffuse
arthralgias specifically in her right shoulder and knees, and a sore throat. On physical
exam she was hypotensive (systolic BP 90 mmHg) tachycardic (pulse 130/min) and tachypneic
(respiratory rate 40/min). She additionally had jugular venous distension of approximately
13 cm and a pulsus paradoxsus of 12 mmHg. Cardiopulmonary examination revealed clear
breath sounds and a very prominent pericardial friction rub. Posterior pharynx was
erthyematous with out exudates. Musculoskeletal exam revealed decreased range of motion
in her right shoulder and tenderness to palpation without evidence of effusion. Laboratory
values: white blood cell count of 51,800, neutrophil count of 97%, sedimentation rate
of 114 mm/hr, c-reactive protein >9, and a ferritin level greater than 100,000. Emergent
echocardiogram was obtained which revealed a large circumferential pericardial effusion
with early diastolic collapse of the ventricles. The patient was taken to the operating
room for an emergent pericardial window placement. A pericardial catheter was placed
and drained 330 ml of serosanguinous fluid which was exudative in nature. Immediately
after the procedure the patients tachycardia resolved, her blood pressure increased
and her dyspnea dramatically improved. All cultures obtained returned without growth
and the patient's antibiotics were discontinued. The patient was placed on high dose
steroids and methotrexate. She reports doing well to date with no further complications.
DISCUSSION
Still's Disease is an infrequent rheumatological disease that until recently was poorly
described in medical literature. It is a systemic inflammatory disorder of unknown
etiology that typically affects young adults. The vast majority of cases present with
a constellation of symptoms reminiscent of a viral syndrome including quotidian fevers,
salmon colored macular rash, sore throat, myalgias, arthralgias, lymphadenopathy,
and serositis. Pleuritis and Pericarditis occur in approximately 25% of patients.
Pleural effusions are usually bilateral and asymptomatic, and thoracentesis often
reveals bloody exudative effusions. Pericarditis is the most alarming, as it may herald
of impending acute cardiac tamponade. Though pericarditis is common, there have been
very few actual reports of cardiac tamponade reported in relation to Still's disease.
This case report allows us the opportunity to discuss the salient features of AOSD
and it's diagnostic criteria. This report also allows us to describe the rare and
potentially fatal complication of cardiac tamponade. This report should enable clinicians
to improve their diagnostic awareness of a complicating illness that presents itself
in many ways.
CAROTID ARTERY STENOSIS AND OCCIPITAL. INFARCTION
T. Thenappan
1; S. Parikh1; P. Kapoor1; D. O'Brien1. 1St. Francis Hospital, Evanston, IL. (Tracking
ID #117447)
LEARNING OBJECTIVES
1. Recognize the anomalous origin of the posterior cerebral artery from the internal
carotid artery. 2. Magnetic resonance imaging (MRI) is superior to computerized tomography
(CT scan) in evaluating cerebrovascular events. 3. Investigate cerebrovascular disease
with both carotid duplex ultrasound and magnetic resonance angiography (MRA) irrespective
of intracranial localization.
CASE
We report a case of a 64-year-old male, with a history of hypertension and diabetes,
who noted sudden onset of a vaguely characterized visual disturbance. The patient
denied similar episodes in the past. The neurological examination was notable only
for a right homonymous hemianopia. Duplex ultrasound of the carotid arteries was suggestive
of high grade occlusion of the left internal carotid artery. The non-enhanced CT scan
of the brain was normal, but MRI revealed an acute infarct of the left occipital lobe,
consistent with examination. The magnetic resonance angiogram showed an anomalous
left posterior cerebral artery originating directly from the left internal carotid
artery (instead of the basilar artery), a hypoplastic A1 segment of the left anterior
cerebral artery, with significant eccentric narrowing of the left internal carotid
artery suggestive of plaque formation. These findings were confirmed by conventional
angiography. The patient subsequently underwent a left carotid endarterectomy.
DISCUSSION
The Circle of Willis, as classically described, is present in only 40% of the population.
The emerging vascular imaging technologies have enhanced our ability to recognize
anatomical variants in the acute clinical setting, and the rational application of
these modalities helps direct appropriate intervention.This case highlights three
major points: First, although an infarct in the posterior fossa due to ipsilateral
carotid artery disease has rarely been reported, the possibility of an anomalous posterior
cerebral artery origin from the carotid artery should be kept in mind. This underscores
the necessity of a complete cerebrovascular evaluation, always including both carotid
studies and magnetic resonance angiography, irrespective of specific intracranial
localization. Second, this case emphasizes the superiority of MRI over CT scan in
the evaluation of cerebrovascular disease. Finally, this report reinforces the importance
of a thorough vascular evaluation in the management of cerebrovascular disease, so
that these anomalies are recognized and, if necessary, factored into medical and surgical
interventional planning.
CAROTID SINUS HYPERSENSITIVITY
M.M. Vasudevan
1; J.T. Bates1; A.A. Taylor1. 1Baylor College of Medicine, Houston, TX. (Tracking
ID #116888)
LEARNING OBJECTIVES
1. Review the clinical presentation and diagnosis of carotid sinus hypersensitivity.
2. Recognize the usefulness of carotid sinus massage (CSM). 3. Understand how to differentiate
the cardio-inhibitory, vasodepressor, and mixed subtypes of carotid sinus hypersensitivity
(CSH).
CASE
A 72 year-old African American man with a history of hypertension and bipolar disorder
was admitted for his second syncopal episode, which occurred while he was seated,
and it was not associated with a prodrome or seizure activity. Pertinent physical
findings included a supine blood pressure of 197/90 and heart rate of 56, without
orthostasis. Carotid exam was normal. Electrocardiogram and telemetry demonstrated
sinus bradycardia and first-degree heart block without arrhythmias. Bedside carotid
sinus massage produced asystole that lasted at least ten seconds. Formal cardiovascular
testing on a tilt table demonstrated a drop in blood pressure from 187/76 (supine)
to 120/70 (with an 80-degree tilt), and there was no associated change in heart rate.
Right-sided CSM produced the same 70 mmHg drop in systolic BP with a concurrent eight
second period of asystole. Left-sided CSM was unremarkable. Exam findings were therefore
suggestive of the mixed subtype of carotid sinus hypersensitivity (CSH). Treatment
was initiated with the placement of a dual chamber cardiac pacemaker.
DISCUSSION
The geriatric population has a high rate of syncope, accounting for up to 3% of emergency
room visits. The differential diagnosis of syncope should include CSH, which is often
overlooked or misdiagnosed. CSM is essential for appropriate identification and differentiation
of the three subtypes of CSH, and it can be performed safely with minimal cardiac
or neurological complications. Appropriate therapy depends on the specific subtype
of CSH. In patients with the cardio-inhibitory subtype, CSM produces at least three
seconds of asystole; pacemaker implantation should abolish this cardio-inhibitory
type. In patients with the vasodepressor subtype, CSM produces a fall in systolic
blood pressure of at least 50 mmHg. The third subtype is a combination of both cardio-inhibitory
and vasodepressor components. CSM during cardiac pacing can differentiate between
pure vasodepressor and mixed types. Therapeutic options for patients with either the
vasodepressor or mixed subtypes include surgical interruption of the afferent or efferent
components of the baroreflex or stripping the vascular adventitia in the area of the
carotid sinus. In summary, a thorough investigation of syncope includes consideration
of CSH, which can safely and effectively be diagnosed with the CSM.
CARPE DIEM
S. Dravid
1; M. Guidry1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane
University, New Orleans, LA. (Tracking ID #117417)
LEARNING OBJECTIVES
1. To stress the importance of a complete differential diagnosis and systematically
to rule out potential causes of mental status changes. 2. Recognize non-convulsive
status epilepticus as a possible cause for mental status changes.
CASE
A 29 year-old man recently started on HAART for HIV presented with four weeks of fever
and confusion. He described one month of night sweats and weight loss. Within three
days of admission, he became completely unresponsive to verbal and physical stimuli.
His vital signs were normal and he had small, but reactive pupils. His reflexes were
normal, and he had no posturing. His electrolytes, liver function tests, CBC, d-dimer,
fibrinogen, coagulation studies, and serum and urine toxicology screens were normal.
A CT of the head showed mild cerebral atrophy with no masses, lesions, or midline
shift. An LP was negative for meningitis. Blood and urine cultures were negative.
He was empirically started on ceftriaxone and vancomycin; his HAART therapy was held.
After no significant improvement, an EEG was obtained that revealed alpha waves consistent
with non-convulsive status epilepticus. A loading dose of phenytoin was administered;
his mental status returned to normal within 24 hours.
DISCUSSION
Altered mental status is a frequently encountered admitting problem. Easily diagnosed
problems such as infection, electrolyte abnormalities, or metabolic encephalopathy
are usually the etiology and should be excluded first. When these are excluded, less
common diagnoses should be investigated. Non-convulsive status epilipticus is an important
cause of altered mental status that is frequently missed because of its lack of symptoms.
Unlike other varieties of seizure disorders, it is frequently not preceeded by an
antecedent seizure history. Once the diagnosis is established by EEG, subsequent studies
should be performed to discover the underlying cause.
CAVITATING LUNG LESION: IT's NOT ALL TUBERCULOSIS
J.S. Nguyen
1; J. Cofrancesco1. 1Johns Hopkins University, Baltimore, MD. (Tracking ID #115854)
LEARNING OBJECTIVES
(1) To recognize the causes of cavitating lung lesion in HIV negative patients, and
(2) to recognize HTLV-1 as a cause of immunosupression.
CASE
A 43 year old gentleman from St Croix without significant past medical history presents
with 4 months of progressively increasing fatigue, weakness, lower extremity swelling,
and shortness of breath; as well as a 30 pound weight loss, low grade fevers and night
sweats. On physical exam, patient was afebrile, had normal vital signs with an oxygen
saturation of 96% on room air, and had a pulmonary exam notable for decreased bibasilar
lung sounds with crackles appreciated in the right mid and upper lung fields. Preliminary
laboratory results were remarkable for WBC = 30,000 with a lymphocyte predominance,
platelets = 114,000, serum calcium = 13.9, LDH = 1375, blood cultures which were sterile
and an HIV antibody which was negative by ELISA. A chest radiograph revealed an ill
defined right upper and middle lobe alveolar infiltrate as well as bilateral pleural
effusions. A chest CT scan revealed a 3.1 × 3.1 cm cavitary mass in the right upper
lobe as well as infiltrates in the right upper lobe, right middle lobe and left upper
lobe. PPD was negative. Bronchoscopy was performed and the bronchoalveolar lavage
was positive for Pneumocystis jiroveci by direct immunofluorescence. The transbronchial
biopsy revealed a focal atypical lymphocytic infiltrate as well as Cytomegalovirus
infection by immunostaining. Upon further testing, the patient was found to be Human
T-cell Lymphotrophic Virus, type 1 (HTLV-1) antibody positive and peripheral blood
flow cytometry revealed a predominantly abnormal T cell population. The combination
of these findings was diagnostic for Adult T-Cell Lymphoma Leukemia and secondary
opportunistic infection with PCP and CMV.
DISCUSSION
Tuberculosis is often considered in a patient with a cavitary lung lesion. However,
a broad differential diagnosis exists and will be discussed including: (1) pulmonary
infections (tuberculosis, atypical mycobacterium, aspergillosis, pneumocystis jeroveci,
lung abscesses, septic emboli), (2) pulmonary infarction, (3) malignancies (lymphoma,
metastatic disease, or primary lung cancer) or (4) vasculitis (Wegener's granulomatosis,
micropathic polyangitis), rheumatoid nodules or sarcoidosis. Given the high calcium
and unusual findings of both pneumocystis jeroveci and CMV pneumonia in a previously
healthy gentleman on no prior medications, immunocompromise was considered. Adult
T-Cell Lymphoma Leukemia (ATLL) will be discussed. ATLL is a peripheral T-cell neoplasm
associated with Human T-cell Lymphotrophic Virus, type 1 (HTLV-1), infection that
is endemic to the Caribbean islands, Japan and Africa. Typical presentation includes
lymphocytosis with an abnormal T-cell population, hypercalcemia, hepatosplenomegaly,
and interstitial pulmonary infiltrates. Patients with ATLL have a functional T-cell
immunodeficiency which predisposes them to opportunistic infections, as this case
has illustrated. Overall prognosis for patients with ATLL is poor with a median survival
of 4 months.
CELLULITIS GONE WILD
B. Weinberg
1; R. Cader2. 1University of California, Los Angeles, Sylmar, CA; 2Sepulveda VA Ambulatory
Care Center, North Hills, CA. (Tracking ID #116311)
LEARNING OBJECTIVES
1. Recognize an unusual presentation of necrotizing fascititis. 2. Recognize the importance
of an early surgical consult when you suspect necrotizing fasciitis.
CASE
A 42 yo white female with a history of intravenous drug use, Hepatitis C, and skin
popping presented to an outside hospital with complaints of abdominal wall cellulitis
and fevers. During her hospitalization there she was afebrile with normal vital signs,
and was described as having a cellulitis on her right abdominal wall. The patient's
cellulitis worsened despite ampicillin/sulbactam and the addition of levofloxacin.
On hospital day 3 the patient was transferred to our facility with a “refractory cellulitis.”
Upon arrival in the late evening the patient was afebrile with stable vital signs
and appeared to be in significant pain related to the local site of infection. On
exam she had an extensive area of erythema, firmness, and induration encompassing
her entire right flank, extending to her hip and into her groin. This area was exquisitely
tender but without creptitance or areas of fluctuance. Antibiotics were continued,
a CT scan was ordered to evaluate for an underlying abscess, and an ID consult was
to be obtained in the AM. The following morning the patient became hypothermic and
hypotensive. Her wound was more erythematous and had spread further down her flank
and groin. She was transferred to the ICU, evaluated by surgery and taken to the operating
room for debridement of necrotizing fasciitis. A 40 × 30 cm area was debrided to muscle
over her right flank. After surgery the patient was continued on broad spectrum antibiotics
and discharged on day 10 with follow-up by Plastic Surgery for a future skin graft.
DISCUSSION
Necrotizing infections of the skin include necrotizing forms of cellulitis and fasciitis.
Necrotizing fasciitis is a deep infection of the subcutaneous tissue that leads to
progressive destruction of fascia and fat. Causative organisms include Group A Streptococcus,
mixed anaerobic/aerobic bacteria and C. Perfrigens. Early recognition of necrotizing
fasciitis is paramount since it can rapidly progress over hours, causing extensive
destruction, toxicity, loss of limb or death. Unexplained pain which increases rapidly
may be the first sign of this process. If untreated this progresses to localized swelling,
edema and tenderness. Later the skin typically becomes dark red with bluish bullae.
These patients may exhibit signs of sepsis and hemodynamic instability seemingly out
of proportion to the lesion. Creptitus on exam and subcutaneous air demonstrated radiographically
are extremely suggestive of necrotizing fasciitis or gas gangrene. Any suspicion of
necrotizing fasciitis requires immediate surgical consultation with potential surgical
exploration and debridement, as well as the initiation of broad spectrum antibiotics.
CEREBROVASCULAR ACCIDENT IN AN ELDERLY PATIENT—IMPORTANCE OF ECHOCARDIOGRAM
S. Dodla
1; T. Townley1; H. Hashish1; Z. Gatalica1. 1Creighton University, Omaha, NE. (Tracking
ID #115697)
LEARNING OBJECTIVES
1) To report a case of Papillary fibroelastoma, a rare cardiac tumor. 2) Recognize
the importance of surgical resection of tumor in symptomatic patients. 3) Emphasize
the importance of echocardiogram in patients with symptoms of CVA.
CASE
Patient is a 73-year-old female who presented with symptoms of numbness of the tongue,
decreased sensation of the mouth and the lips along with difficulty swallowing, chewing
and talking. Her other significant past medical history included Coronary artery disease,
Hypertension and Hyperlipidemia. She was found to have Paroxysmal atrial fibrillation
during the stay in the hospital. MRI and MRA of head showed a focal acute infarct
in the right posterior frontal lobe with an old ischemic infarct in the left lenticular
nucleus. Trans Thoracic Echocardiography showed no evidence of mass or thrombus. Trans
Esophageal Echocardiography (TEE) revealed a 1.3 × 1.1 cm mobile, broad based echo
density on the ventricular surface of the right coronary cusp of the aortic valve
with trace central aortic regurgitation. The mass was surgically excised and the diagnosis
of PF was histologically confirmed. Follow up TEE revealed no residual tumor and no
aortic regurgitation. The patient did not have any embolic episodes for a follow up
period of twelve months.
DISCUSSION
PF is a rare, benign, slowly growing cardiac tumor. These tumors have been reported
sporadically in live patients since the introduction of echocardiography especially
TEE, during evaluation of patients with transient ischemic attack, cerebrovascular
accident and myocardial infarction. Although this patient had paroxysmal atrial fibrillation
it was deemed necessary to surgically remove the aortic valve mass to prevent recurrent
strokes, as the possibility of the latter being the source of embolus was high. Although
PF may possess some characteristic echocardiographic features, histopathologic evaluation
is essential to differentiate them from other intra-cardiac benign and malignant tumors.
CHEST PAIN IN A YOUNG MAN: SPONTANEOUS PNEUMOMEDIASTINUM
T. Sai
1. 1John H. Stroger Hospital of Cook County/Rush University Medical Center, Chicago,
IL. (Tracking ID #115900)
LEARNING OBJECTIVES
1. Recognize spontaneous pneumomediastinum as a cause of chest pain in young individuals.
2. Review the clinical features, diagnosis and treatment of spontaneous pneumomediastinum.
CASE
A 17 year-old man presented to the emergency department with the acute onset of substernal
chest pain . The patient was well until the afternoon of admission when he developed
the sudden onset of sore throat one hour after eating lunch. He then developed sharp
substernal chest pain followed by anterior neck stiffness. There was no history of
trauma, dyspnea, cough, dysphagia or ingestion of sharp objects. On examination, his
vital signs were normal. There was subcutaneous crepitation of the neck, supraclavicular
fossae and anterior chest wall. Cardiac examination was normal as well as the remainder
of the physical examination. Chest and neck x-ray revealed subcutaneous linear lucencies
without evidence of free mediastinal air. CT scans of the neck and chest showed free
mediastinal air with dissection into the posterior pharynx. All mediastinal structures
were normal. Fiberoptic laryngoscopic evaluation and contrast esophagram were normal.
A diagnosis of spontaneous pneumomediastinum was made. The patient was treated with
ibuprofen for analgesia and bed rest. The patient's symptoms resolved after 48 hours.
A repeat chest CT scan showed reduction in mediastinal air. To date, the patient has
had no recurrence of symptoms.
DISCUSSION
Spontaneous pneumomediastinum is the non-traumatic presence of free air in the mediastinum.
Although it is second only to spontaneous pneumothorax as the admitting diagnosis
for healthy young people with the sudden onset of chest pain or dyspnea, it occurs
in only 1 in 13,000 young persons presenting with chest pain. It results when alveoli
rupture in the absence of trauma, causing escaped air to dissect along the interstitial
bronchovascular sheath into the mediastinum. The Valsalva maneuver and illicit drug
use are predisposing factors. Patients most commonly experience chest pain; other
complaints include dyspnea, dysphagia and throat discomfort. Findings on physical
examination include subcutaneous emphysema and Hamman's sign, a cardiac systolic crunch
best heard at the apex. Chest x-ray may reveal the presence of free air in the mediastinum
as well as subcutaneous emphysema. CT imaging confirms the diagnosis and helps evaluate
mediastinal structures. Esophageal imaging to rule out rupture is sometimes warranted.
Management includes symptom control and observation. Most patients have resolution
of symptoms within 2 to 7 days and seldom require further intervention. Although uncommon,
recurrences have been reported. Awareness of this rare, benign entity may help avoid
unnecessary invasive tests and procedures.
CHRONIC ALCOHOLISM PRESENTING AS SEVERE ACUTE AXONAL NEUROPATHY
G.G. Tapia
1; L.D. Stagner1; L.M. Tapia1. 1Henry Ford Hospital Detroit, Detroit, MI. (Tracking
ID #115809)
LEARNING OBJECTIVES
Recognize acute cases of alcohol-related polyneuropathy and distinguish clinical features
between guillain-barre syndrome and alcohol-related acute axonal polyneuropathy
CASE
A 33 year-old female with a four-year history of alcohol abuse was admitted to the
hospital with generalized ascending weakness and numbness for three weeks. The patient
denied flu-like or gastrointestinal symptoms. A physical exam revealed diminished
power 3/5 of the extremities, decreased sensation to pinprick, pain, and temperature
distally, areflexia, flaccid muscle tone, dysphagia and a vital capacity (VC) of 900cc.
RBC folate, thiamine, and urine methylmalonic acid levels were normal. The CSF revealed
normal protein without pleocytosis, a negative VDRL, and the absence of CMV DNA, Lyme
antibodies, cryptococcal antigen, and oligoclonal bands. TSH, ANA, anti SS-A, anti
SS-B and RF were all normal. HIV was non-reactive. Additional studies including anti-Hu
antibodies, VZV IgM, EBV capsid IgM, HTLV-1and 2, and a viral hepatitis screen were
all unremarkable. Urine and stool porphyrins were unremarkable. A 24-hour urine showed
normal lead, thallium, aluminum, and mercury levels. A serum ganglioside antibody
panel was negative and ceruloplasmin level normal. An EMG revealed an axonal sensorimotor
polyneuropathy with signs of acute denervation and reinnervation. The patient did
not require 20. Following abstinence from alcohol, her motor strength, deep tendon
reflexes, and VC improved.
DISCUSSION
Approximately 9% of patients with chronic alcohol abuse suffer from insidious peripheral
neuropathy. The few reported cases of acute alcoholic neuropathy (AAN) describe onset
of symptoms to be from few days to several weeks. This entity must be distinguished
from acute polyneuropathies such as Guillain-Barre syndrome (GBS). Severe sensory
loss is uncommon in classic GBS but frequent in axonal GBS. Unlike any GBS patient
with severe tetraparesis, cranial nerve abnormalities and respiratory insufficiency
were less severe in our patient. Less than 5% of GBS cases in North america are primary
axonal forms. Although autoimmunity in GBS is debatible, antibodies to gangliosides
(GM1, GM1b, GD1a) correlate with the presence of axonal GBS. All patients with AAN
may regain their ability to walk within months of alcohol abstinence as opposed to
15% of GBS patients who are unable to walk after one year of diagnosis. The pathophysiology
of AAN is still uncertain. Vitamin levels and nutritional state poorly correlate with
polyneuropathy; however, preliminary data describe the direct toxic effect of alcohol
in peripheral nerves. Alcohol abstinence is the treatment of AAN while immunotheraphy
is probably unnecessary compared to cases with GBS.
COLA-COLORED URINE IN VIRAL SYNDROME: RHABDOMYOLYSIS WITH A CPK OF OVER ONE MILLION
UNITS WITHOUT ACUTE RENAL FAILURE
G.T. Arbour
1; L. Mitchell1; R. Palmer1; D. Strain1; J. Huang1. 1Louisiana State University Medical
Center at Shreveport, Shreveport, LA. (Tracking ID #115650)
LEARNING OBJECTIVES
1. Recognize that viral illness and flu-like syndromes can cause severe rhabdomyolysis.
2. Recognize that renal failure can be prevented in rhabdomyolysis if diagnosed and
intervened early.
CASE
A 20-year old previously healthy black male presented with the complaint of “cola-colored
urine” for one day. 7 days prior to presentation, he developed fever, headache, dry
cough, rhinorrhea, sore throat and general malaise. 5 days later, he developed severe
myalgias and muscle weakness, especially in his thighs, shoulders and back, followed
by reddish-brown colored urine. The patient denied alcohol or cocaine use, recent
strenuous activity, or trauma. Physical exam was unremarkable except for diffuse tenderness
of most muscle groups. Motor strength of all extremities was also decreased at 4/5.
UA on admission demonstrated large blood on dipstick with 0–2 RBCs on microscopy.
Serum creatine phosphokinase (CPK) revealed a value of 395,800 U/L which peaked 3
days later to a maximum of 1,213,650 U/L. Elevated AST and ALT paralleled CPK in kinetics;
however, alkaline phosphatase remained normal. Urine myoglobin peaked at 162 ng/mL.
A UDS was negative for cocaine. Electrolytes were normal and a viral hepatitis panel
and ANA were negative. Nasal aspirate was negative for influenza by ELISA. Upon diagnosis,
IV fluid with bicarbonate was promptly initiated in the ED. After 3 days, the CPK,
transaminases, and myoglobin began to trend down until they normalized. The serum
creatinine remained normal. The patient's symptoms resolved and he was discharged
home a week later.
DISCUSSION
Rhabdomyolysis is commonly caused by drugs, alcohol, seizures, and trauma. It has
also been reported in viral and bacterial infections. Influenza is the most common
viral etiology. The pathophysiology underlying virus-induced muscle damage is unclear.
Two mechanisms have been postulated: direct viral invasion and myotoxins mediated
by cytokines. Viral syndrome seems to be responsible for this case based on the patient's
symptoms and exclusion of other etiologies. Influenza is likely, although not confirmed,
given the epidemic season and several confirmed local cases. The highest CPK in influenza
was reported at over 500,000 U/L and nearly half of the patients developed acute renal
failure (ARF). Although CPK remains the most reliable indicator of muscle damage,
it does not always correlate with the development of ARF. This case demonstrates that
even a CPK value of over 1.2 million U/L does not necessarily lead to ARF. With a
high index of suspicion during flu season, diagnosis of rhabdomyolysis can be made
by history, physical exam, and simple lab tests. Early intervention with aggressive
hydration is the key to the prevention of ARF.
COLCHICINE OVERDOSE — NOT JUST DIARRHEA
B.P. Sankarapandian
1; S.K. Thambidorai1; M. Bandara1; S. Dodla1; L. Morrow1. 1Creighton University, Omaha,
NE. (Tracking ID #115553)
LEARNING OBJECTIVES
1. Demonstrate the classic pattern of the clinical course after colchicine overdose.
2. Recognize the current modalities in managing colchicine overdose. 3. Recognize
the fatal potential of a common drug.
CASE
23-year-old male with history of depression was admitted to the intensive care unit
after ingesting 60 mg of colchicine. He initially presented to a rural hospital where
he underwent gastric lavage followed by activated charcoal administration. The patient's
initial symptoms upon presentation to our hospital included weakness, lethargy and
nausea. During the course of the hospitalization the patient developed an upper gastrointestinal
bleed (GI) with subsequent hypotension and anuria. The patient's condition worsened
when he became progressively more dyspneic eventually leading to acute respiratory
distress syndrome (ARDS). Hepatic failure ensued with the following enzymes values:
alk phos 929 IU/L, AST 1466 IU/L, ALT 366 IU/L, and T bili 2.6 mg/dL. He later developed
rhabdomyolysis with a serum myoglobin of 480 ng/mL, urine myoglobin 5100 mg/L, CPK
16260 IU/L, BUN 38 mg/dL, and serum creatinine of 3.6 mg/dL. His white blood cell
count was initially high, however he later developed signs of agranulocytosis. The
serum colchicine level was noted to be 12 ng/mL on admission. The patient underwent
continuous renal replacement therapy to try and correct his metabolic abnormalities,
but despite the supportive management the patient died 5 days after the suspected
time of ingestion. The patient's death was secondary to multi-organ failure.
DISCUSSION
Colchicine overdose is a rare occurrence. Fatalities have been reported after ingestion
of just 7 to 12 mg. The morbidity and mortality are directly related to the dose ingested.
Toxic manifestations appear after a delay of 2 to 12 hours following ingestion. Symptoms
progress in 3 stages. Stage I (Days 1–3) includes a GI phase (Nausea, vomiting, abdominal
cramps, diarrhea) and a circulatory phase (hypovolemia, hypotension and cardiogenic
shock). This is followed by hypoventilation and acute respiratory distress syndrome.
Stage II (Days 3–10) includes bone marrow aplasia, coagulation disorders, polyneuritis,
myopathy, and acute renal failure. Stage III: (10 + days) is the recovery phase. There
is no specific antidote for colchicine and the only treatment available is supportive
management. There are case reports of patients treated with an antibody made from
goat serum. This antidote is not readily available and is no longer produced.
CONJUNCTIVITIS, COUGH AND FEVER IN A NEPALI MOUNTAIN PORTER
H.K. Liss
1. 1University of Washington, Seattle, WA. (Tracking ID #116042)
LEARNING OBJECTIVES
1) Recognize the clinical features of measles, particularly prior to the development
of exanthem. 2) Assess and manage the public health issues posed by a measles outbreak
in a population with low levels of passive and active immunity. 3) Identify and treat
measles and the associated complications.
CASE
An 18 year old male Nepali mountain porter presented to a new healthcare post in the
Everest region of Nepal with a chief complaint of red eyes, malaise, coryza, anorexia
and cough of 3 days duration. Physical exam revealed fever of 39.8°C, tachycardia
of 110 bpm, significantly injected conjunctiva bilaterally, and several 1–3 mm white
spots on the posterior buccal mucosa bilaterally. The rest of the exam was significant
only for bibasilar crackles and the absence of rash. Concern for prodrome of measles
prompted immediate isolation of the patient in a room in the trekking lodge where
the clinic was situated. Initial management also included oral hydration, anti-pyretics,
and antibiotics for possible measles pneumonia with bacterial super-infection. By
the following morning, a blanching erythematous macular-papular rash erupted over
the patient's entire body, sparing his palms and soles. Generally such a patient would
be evacuated on foot, by porter or yak to the permanent hospital 5–7 hours away for
further management. However, a scheduled immunization day at the hospital precluded
such an evacuation, which would possibly expose many unimmunized children and adults
to measles. Thus, a porter walked to the hospital, notified the staff of the patient's
illness, and requested doses of measles vaccine for contacts. When the porter returned
with 10 doses of measles immunization, those Nepalis assessed to be at highest risk
of contracting measles were administered vaccine. On the following day, the patient
was escorted to the hospital and kept in respiratory isolation for a total of 5 days
after the initial appearance of the rash. The patient recovered, returning to work
as a porter the next week. In the following month, approximately 10 additional suspected
cases of measles were seen at the hospital; however, none of these cases had known
contact with this patient.
DISCUSSION
This case underscores the importance of early diagnosis of highly communicable diseases
in populations with low levels of immunity. In the case of measles, making the diagnosis
and isolating the patient prior to development of rash may help stem epidemics. In
addition, the case highlights geographically specific issues to the Himalayas, as
conjunctivitis and cough are very common complaints, posing diagnostic confusion with
the presentation of prodromal measles. The challenge of managing both patient and
population during an outbreak of measles in remote regions which are resource-poor,
difficult to access, and fraught with crowded living conditions emphasizes the need
for improved primary prevention through immunization.
CONSERVATIVE MANAGEMENT OF CHRONIC MESENTERIC ISCHEMIA
D.N. Goldson-Prophete
1. 1University of Medicine and Dentistry of New Jersey, Newark, NJ. (Tracking ID #102070)
LEARNING OBJECTIVES
1. Recognize the signs/symptoms of chronic mesenteric ischemia. 2. Recognize treatment
options for mesenteric ischemia.
CASE
Mr. S is a 61-year-old man who complained of sharp “stabbing” mid-epigastric abdominal
pain radiating towards his umbilicus for approximately one year. He further complained
of decreased appetite with avoidance of meals and a 30lb. weight loss. He denied any
correlation between the onset of pain and his position or activity. His past medical
history is significant for diabetes, hypertension, hyperlipidemia, atrial fibrillation
(AF), coronary artery disease, and congestive heart failure (CHF) with an ejection
fraction of ~15%. His current medications include Esomeprazole, Glyburide/Metformin,
Metoprolol , Pravastatin, Losartan, Furosemide, Warfarin and enteric-coated Aspirin.
No significant family history. He denies tobacco, alcohol. On physical examination,
Mr. S was an obese male. Bowel sounds were normoactive and abdomen was soft with mid-epigastric
tenderness. No rebound or guarding. No abdominal bruit. Rectal examination was normal
and he was guiaic negative. CT scan of the abdomen and pelvis was notable for focal
narrowing of the superior mesenteric artery and mural calcification. Subsequent angiography
revealed mild to moderate stenosis of 50–60% in the proximal portion of main superior
mesenteric artery (SMA). However, it was not flow limiting and there was no pressure
gradient across the stenosis. The patient subsequently underwent dilatation and stenting
of his SMA with reduction of the stenosis to 10–20%, after which he reported complete
relief of his symptoms.
DISCUSSION
Mesenteric ischemia due to chronic arteriosclerotic occlusive disease usually results
in the gradual development of postprandial pain associated with avoidance of meals
and resultant weight loss. Diagnosis is based on clinical symptoms and arteriographic
demonstration of an occlusive process of the splanchnic vessels. Endarterectomy or
bypass procedures of the celiac and/or SMA have shown the greatest success in treating
this condition. However, percutaneous ballon angioplasty with/without stenting, becomes
the procedure of choice for high-risk patients in whom a more conservative approach
is warranted, such as Mr. S. The literature has generally concentrated on the management
of those patients with a “severe” stenosis of one or more of the splanchnic vessels.
This case demonstrates that even a “moderate” stenosis of the SMA can be clinically
significant and may warrant an attempt at revascularization. A prospective trial of
endarterectomy vs. percutaneous angioplasty in these patients (as opposed to those
with severe stenoses) has not been done.
CRACKING THE NECK CAN CAUSE A STROKE
F.K. Salahuddin
1; M. Gaddamanugu2; S.G. Nace3. 1University of Illinois College of Medicine at Peoria,
Peoria, IL; 2University of Illinois College of Medicine Peoria,SFMC, Peoria, IL; 3University
of Illinois College of Medicine, Peoria,SFMC, IL, IL. (Tracking ID #117189)
LEARNING OBJECTIVES
1. Sudden repeated neck movements can cause a vertebrobasilar artery dissection. 2.
Thorough history taking is the key towards an accurate diagnosis. 3. Isolated episodes
of vertigo may be the only manifestation of vertebrobasilar ischemia.
CASE
This was a 51 year old white male who presented to the ED with sudden onset of vertigo,lightheadedness
and severe right sided headache following sudden neck movement. He closed his eyes
in an attempt to stop the vertigo with out much relief, he then described his gait
as preferential tilting to his right side. Patient required assistance in getting
out of the car because of his leg weakness. Symptoms occurred as the patient “popped”
his neck as he was in the habit of doing it daily, and especially that day he repeatedly
“popped” his neck many times in an attempt to “pop” into place. Past medical history
was significant for neck pain for which patient had consulted chiropractor; his last
visit being was a few months ago. Upon arrival his neurological exam was unremarkable.
Initial non contrast CT of the head and lumbar puncture were normal. MRI/MRA demonstrated
right vertebral artery dissection at C1 vertebral level with infarction in the distribution
of the PICA. These finding were consistent with dissection and frank occlusion of
right vertebral artery. Patient was successfully treated with clopidogrel due to his
allergy to aspirin. He had complete recovery. He was however advised to avoid rapid
neck movements as well as manipulations.
DISCUSSION
Concerns about vertebrobasilar artery dissection after chiropractic as well as self
manipulation are common. Anatomical variations of the vertebral arteries and their
branches are not infrequent and may constitute a predisposing factor to complications
after neck manipulation. Vertebrobasilar artery dissection is a reported complication
of sudden neck movements. There are reported cases in the literature as a result of
chiropractic manipulation, blunt trauma to the neck, and in sports requiring sudden
and rapid movements. Neurological symptoms may improve depending upon the severity
of the injury. Long-term anticoagulation is the recommended. A careful history in
the proper setting is essential to a diagnosis and can prompt the clinicians to do
appropriate diagnostic study.
CULTURAL ISSUES IN PRIMARY CARE MANAGEMENT OF RUSSIAN IMMIGRANTS
B.H. Warren
1; I. Pines2. 1Denver Health and Hospital Authority, Denver, CO; 2University of Colorado
Health Sciences Center, Denver, CO. (Tracking ID #116362)
LEARNING OBJECTIVES
1. Become familiar with the cultural conditions of Russian immigrants that may interfere
with succesful management of chronic disease for these individuals. 2. Apply knowledge
of cultural barriess to solving clinical problems among Russians with chronic disease.
CASE
68 YO Russian speaking engineer who immigrated to the US in 1999. He has CAD, diabetes
and uncontrolled hypertension. His BP is 195/100, HgA1C is 11.8, LDL cholesteral is
155 and he is chronicall depressed. He has no concept of the physiology and pathogenesis
of hyperlipidemia and takes previously prescribed blood pressure medicines “PRN” for
not feeling well. Dietery instructions given him earlier in English were not understood.
(On or two other vignettes will be offered to illustrate the issues addressed)
DISCUSSION
BACKGROUND Federal mandates and National Quality Guidelines and Policies require that
practitioners address cultural competence for special populations in the care of our
patients. Healthy People 2010 sets goals for reducing cultural disparities in preventive
management of diseases. Cultural issues have a significant impact on the successful
management of chronic diseases according to guidelines and expectations when measuring
the clinical performance of general internists. Addressing these goals requires explicit
knowledge of the cultural issues, barriers and opportunities that are relevant for
each of the diverse old and new populations seeking health care services in the United
States. Newer immigrants are more vulnerable as they bring foreign experience and
expectations of medical health care with which American providers may be unfamiliar.
One such population about whom little has been published is that of recent Russian
immigrants. DISCUSSION Examples of Russian cultural barriers and opportunities addressed
in this presentation include attitudes towards American medicine, skepticism about
preventive health care, reluctance to use medications regularly, and fear of chronic
disease and hospitalization. High expectations for absolute cure of chronic illness,
religious interventions (in some populations), and free access to medical care add
to complicating variables. Underlying high prevalence of chronic depression, previous
adverse experiences with chronic disease and poor availability of medications also
require special consideration.
CUSHING's SYNDROME DUE TO A PULMONARY CARCINOID
E.H. Orth
1; T. Dorsch1; L. Cation1. 1University of Illinois at Peoria, Peoria, IL. (Tracking
ID #116346)
LEARNING OBJECTIVES
Recognize the clinical features of Cushing's Syndrome. Recognize the classic features
of a pulmonary carcinoid tumor.
CASE
A 40 year-old man with a 3-year history of congestive heart failure due to a presumed
viral cardiomyopathy presented with hypokalemia despite vigorous oral potassium replacement.
He also noted increasing proximal muscle weakness. On physical exam, plethora, striae,
bruising, muscle wasting, and central obesity were present. Analysis revealed the
potassium on admission was 2.0 mmol/L. A 24-hour urine free cortisol was 350 mcg/24
hours (normal <50). There was no suppression of urinary free cortisol with dexamethasone
2 mg po every 6 hours for 48 hours. CRF testing showed baseline serum cortisol was
31 mcg/dl and peak cortisol was 34 mcg/dl; baseline plasma ACTH was 52 pg/ml and peak
was 64 pg/ml. A chest film showed healing rib fractures. Further imaging with CT scan
of the chest showed a small infiltrate in the left upper lobe thought to represent
inflammatory changes. Petrosal sinus sampling for ACTH did not demonstrate a gradient.
An octreotide scan showed uptake in the left lung, corresponding to the infiltrate
on CT scan. A carcinoid tumor was resected from the left lung. Immunoperoxidase staining
of the tumor was markedly positive for ACTH.
DISCUSSION
Bronchial carcinoid tumors comprise approximately 2% of all ectopic ACTH secreting
neoplams, and 3.5% of all carcinoid tumors. Patients with bronchial carcinoid are
less likely to have flushing or diarrhea than carcinoid tumors from the embryonic
midgut or hindgut. Ectopic ACTH secrection and right-sided heart disease can be features
of carcinoid syndrome. Typically these tumors pursue an indolent course, with onset
of symptoms preceding diagnosis by a mean of 4.5 years. In summary, our case represents
a patient with a history of heart disease and Cushing's Syndrome due to a pulmonary
carcinoid tumor.
CYCLIC FEVER IN A PREGNANT WOMAN
T.T. Tran
1; L.B. Lu2. 1Baylor College of Medicine, Houston, TX; 2Baylor College of Medicine,
Friendswood, TX. (Tracking ID #117249)
LEARNING OBJECTIVES
1) Construct a differential diagnosis for cyclic fever in pregnant women. 2) Recognize
malaria as the cause of cyclic fever in a patient from an endemic area. 3) Review
relapse, perinatal outcome, and treatment of Plasmodium vivax infection during pregnancy.
CASE
A 19-year-old G2P1 woman, 21 weeks pregnant, presented to the emergency room with
10 day history of cyclic fever and chills. She was in her usual state of health until
10 days prior to presentation when she developed intermittent shaking chills and fever
up to 104 F. Each episode would last an hour, usually in early morning and late afternoon.
The patient denied cough, abdominal pain, diarrhea, dysuria, and weight loss. She
was from El Salvador and immigrated to the United States 6 months ago. On arrival,
her vital signs were with temperature of 101.4, blood pressure of 90/40 with no orthostatic
hypotension, heart rate of 105, and respiration rate of 18. She had pale conjunctiva,
supple neck, clear lungs, normal heart sounds, gravid abdomen with no tenderness,
no costovertebral angle tenderness, and no palpable lymphadenopathy. Blood work revealed
WBC 7.7 with 68% neutrophils and 21% lymphocytes, Hgb 11.1, Hct 31.3, platelets 146,
normal chemistries, and normal liver function tests. LDH was 329. Urine and blood
cultures were negative. A peripheral blood smear revealed multiple red blood cells
with rings consistent with malaria. Pathology confirmed that the morphology was consistent
with Plasmodium vivax in different stages of development. The patient was treated
with oral chloroquine with resolution of her fever. After her delivery, primaquine
was started to eradicate the liver stages of Plasmodium vivax.
DISCUSSION
In a pregnant woman, cyclic fever has a limited differential diagnosis which includes
cyclic neutropenia, lymphoma, and malaria. Malaria should be considered in a patient
with cyclic fever from an endemic area. The initial infection with P. vivax can present
with mild or no symptoms. However, P. vivax produces dormant liver stages that may
cause late relapse 6 to 11 months after the initial infection. Pregnancy predisposes
to relapse due to depressed cell-mediated immunity and sequestration of parasites
in the placenta. P. vivax infection during pregnancy is associated with maternal anemia
and decreased fetal birthweight. The preferred drug during pregnancy is chloroquine.
For chloroquine-resistant malaria, quinine combined with clindamycin is the treatment
of choice. Primaquine is used after delivery to eradicate latent liver stages of P.
vivax. In conclusion, malaria should always be in the differential in all synchronized
cyclic fever.
DEEP VENOUS THROMBOSIS SECONDARY TO DEEP VENOUS THROMBOSIS PROPHYLAXIS
K. Paranada
1; M. Lim1. 1University of Connecticut, Farmington, CT. (Tracking ID #116572)
LEARNING OBJECTIVES
Diagnose and manage deep venous thrombosis (DVT) secondary to heparin-induced thrombocytopenia
as a complication of heparin for DVT prophylaxis.
CASE
A 72 year old female with a history of hypertension, type 2 diabetes mellitus and
obesity, underwent bilateral knee replacement for osteoarthritis. She was given enoxaparin
for DVT prophylaxis and was discharged stable on her fourth post-operative day. She
returned 6 days later complaining of left leg pain localized below the knee. Examination
showed the leg to be markedly swollen, cold, pale, with weak pulses. The patient was
immediately taken for fasciotomy of the involved leg where there was note of muscle
edema but no hematoma or myonecrosis. Pedal pulses improved. Laboratory data showed:
platelet of 13,000/ml3 (normal on discharge), PT of 17.7, INR of 2.42 and PTT of 30.
However, a few hours post-fasciotomy, the left leg was noted to be progressively more
mottled, cyanotic and edematous. The patient was started on IV lepirudin. Venous angiogram
showed massive DVT involving the left common femoral vein down to the popliteal vein.
Thrombolytic therapy using urokinase for limb salvage was started. An IVC filter was
also placed. INR was kept between 2 to 3 with lepirudin.
DISCUSSION
Heparin has long been considered the standard in DVT prophylaxis. However, complications
arising from heparin therapy, such as that seen in this patient, can occasionally
be life or limb threatening. Type II heparin-induced thrombocytopenia occurs in 3%
of patients receiving heparin for 5 or more days. The major target antigen is a multimolecular
complex of platelet factor 4 and heparin. Immune complexes interact with the platelet
Fc gamma II receptor, which leads to platelet activation, formation of prothrombotic
microparticles, and generation of thrombin. The key in diagnosis of HIT is a high
index of suspicion as assays with high sensitivity and specificity may not be readily
available. The appearance of otherwise unexplained thrombocytopenia, thrombosis associated
with thrombocytopenia, or even a normal platelet count which has fallen 50 percent
or more from a prior value should raise the possibility of HIT in any patient begun
on heparin therapy within the preceding five to ten days. Once HIT is clinically suspected,
therapeutic management consists of removal of the immune stimulus, by discontinuing
heparin therapy and inhibition of thrombin, either directly (lepirudin and argatroban)
or by blocking the generation of new thrombin (danaparoid). In the face of thrombosis,
thrombolytic therapy may also be considered. Thus, the decision to put patients on
heparin comes with the responsibility and vigilance of screening for this important
complication.
DEMENTIA WITHOUT MEMORY LOSS?
P. Koneru
1; G. Prakash1; R.D. Hobbs1. 1Oakwood Healthcare System, Dearborn, MI. (Tracking ID
#117109)
LEARNING OBJECTIVES
To recognize the existence of atypical dementias that are language based and without
significant memory loss.
CASE
A 71-year-old man accompanied by his wife presented for the evaluation of dementia.
His history was significant for hypertension and hyperlipidemia for which he took
benazepril and lovastatin. He was healthy until five years previously when he noted
word-finding difficulties. He could now only express himself in monosyllables. There
were no focal neurological deficits. His behavior, mood, ability to recall recent
events and recognize people were intact. He had mild difficulty dressing. Social graces,
facial expressions, coordination and gait were normal. He followed commands. He accurately
drew a clock and wrote a sentence without spelling errors. His MMSE was measured at
10/30. CBC, metabolic panel, Vitamin B-12, Folate, TSH, Ceruloplasmin levels, and
HIV and Syphilis serologies were normal. CT and MRI only showed generalized cerebral
atrophy. Neuropsychological testing revealed Primary Progressive Aphasia (PPA).
DISCUSSION
Unlike most common dementias where memory problems are common and an integral part
of the diagnosis, PPA is an atypical dementia characterized by a relentless dissolution
of language with relative preservation of memory. Patients usually present with word-finding
difficulties, spelling errors or abnormal speech patterns. Unlike Alzheimer's disease,
these patients can recall and evaluate recent events despite an inability to express
their knowledge verbally. Folstein's MMSE because of its reliance on verbal expression
overestimates the degree of the patient's cognitive dysfunction in PPA and may lead
to inappropriate labeling and ineffective treatment. Neuropsychological testing is
the diagnostic modality of choice. There is no effective pharmacological treatment
for PPA although speech therapy is useful in exploring alternative communication strategies.
Explaining the nature of the condition is one of the greatest benefits in terms of
coping with the impairment and recognizing the primary problem to be expressive rather
than cognitive.
DEPRESSION AS A RISK FACTOR FOR HYPOGLYCEMIA IN A WELL-CONTROLLED DIABETIC
M.A. Mendiola
1; E. Coffey1. 1Hennepin County Medical Center, Minneapolis, MN. (Tracking ID #117183)
LEARNING OBJECTIVES
1) Recognize that diabetics have a higher incidence of depression than non-diabetics.
2) Recognize that depression is associated with poor glycemic control. 3) Recognize
that anorexia associated with depression may put a diabetic a risk for hypoglycemia.
CASE
A 71 year old female with a history of type 2 diabetes presented to her primary physician
for routine follow up. She had been maintained on a regimen of NPH and regular insulin
with a recent glycohemoglobin of 6.3%. She had previously experienced few hypoglycemic
reactions, but the episodes had become more frequent over the previous two months.
At this visit, she also described symptoms consistent with depression, including anorexia
and poor motivation for meal preparation. Venlafaxine was initiated, and her insulin
regimen was switched to Glargine and Lispro, to allow more flexibility. She was specifically
instructed not to take her short acting insulin if she was not eating. However, she
continued to have frequent serious hypoglycemic events requiring emergent medical
care. Her insulin regimen was significantly liberalized until her depression could
be brought under better control.
DISCUSSION
It has been shown in a meta-analysis by Anderson et al. in Diabetes Care, 2001, that
diabetics are twice as likely as non-diabetics to suffer from depression (14–26% in
diabetics vs. 5–9% in non-diabetics, with an overall odds ratio of 1.9). Depression
is also associated with poor glycemic control, increased microvascular and macrovascular
complications, as well as increased healthcare expenditures. Total healthcare expenditures
for people with depression and diabetes were 4.5 times as high as for those with diabetes
without depression, in a 2002 study by Egede et al. in Diabetes Care. This case demonstrates
the need for close follow up in diabetics diagnosed with depression. Moreover, it
may be necessary to tolerate hyperglycemia in the short run to reduce the risk of
life-threatening hypoglycemia until the depression can be controlled. Further research
is necessary to investigate the risk of hypoglycemia in diabetics with depression.
DIABETES INSIPIDUS COMPLICATING GASTRIC BYPASS SURGERY
D.L. Mercado1; P. Liew
2. 1Baystate Medical Center, Wilbraham, MA; 2Baystate Medical Center, springfield,
MA. (Tracking ID #116467)
LEARNING OBJECTIVES
To appropriately screen preoperative gastric bypass surgery patients for Diabetes
Insipidus
CASE
A 26 year old woman with morbid obesity was admitted for gastric bypass surgery. Her
past history was notable only for polycystic ovarian syndrome and depression. On postoperative
day 1, she had a high urine output, but had clinical signs of volume depletion. She
complained of thirst but was unable to take adequate oral fluids due to intake limits
from the postoperative gastric bypass diet. Her initial serum sodium was 151, but
her urine specific gravity was 1.006; renal function was normal. IV fluid resuscitation
was initiated resulting in urine output of 7 liters in 24 hours. She was suspected
of having Diabetes Insipidus. History from the patient's mother revealed chronic polydipsia
and polyuria, which the patient had failed to mention. Further labs showed serum Na
149, urine sodium 140, and serum osms 302. A trial of vasopressin decreased her urine
output dramatically from 300 cc/hr to 50 cc/hr with a > 100% increase in urine osms.
Work up revealed no specific cause and the condition was deemed idiopathic central
DI. With daily vasopressin nasal spray she was able to maintain adequate hydration
while adhering to her post-surgical fluid limits, and her polyuria and polydipsia
resolved.
DISCUSSION
Central DI is a rare neurohypophyseal disease defined as polyuria of 2–10 L/day with
dilute urine (SpGr 1.000–1.005) in conjunction with high serum osmolarity and high
serum sodium. The most common causes are trauma or neurosurgery, but 30–50% are idiopathic.
Our patient had daily symptoms consistent with DI which she did not think to mention
preoperatively because of their chronicity. Postoperatively undiagnosed DI can result
in severe volume depletion because of limited access to free water. This is particularly
true of gastric stapling and gastric bypass patients, who have limited oral intake
allowances, especially during their first few days postoperatively. Preoperative screening
for DI is critical in these patients since they would not be able to maintain hydration
without IV fluids due to their limited oral intake postoperatively. In fact, the presence
of nephrogenic DI, which is poorly responsive to vasopressin, is an absolute contraindication
to this type of surgery. Preoperative patients should be questioned about polyuria
and polydipsia. If symptoms are present and not due to hyperglycemia, urine and serum
osms, and urine and serum sodium should be checked. If the clinical findings and history
are suspicious, water deprivation testing can be considered to confirm the diagnosis.
DIAGNOSIS AND MANAGEMENT OF A PREGNANT FEMALE WITH BRUISES AND SPONTANEOUS ABORTIONS:
A CATCH 22
J. Cunningham
1; M. Panda1; W.P. Caine1. 1University of Tennessee, Chattanooga, Chattanooga, TN.
(Tracking ID #115585)
LEARNING OBJECTIVES
1. Recognize the effect of pregnancy on platelet aggregation disorders 2. Discuss
the diagnosis, categorization, and treatment of thrombophilic disorders 3. Recognize
the dilemma in the evaluation and treatment of combined hypercoagulable and hypocoaguable
disease.
CASE
A 19 year old black female G4P0030, at 9 weeks of gestation presented with spontaneous
bruising. She was diagnosed with von Willebrand factor deficiency 3 months ago. She
has a history of menorrhagia but denies history of bleeding from other sites. No family
history of bleeding disorders. She has had two spontaneous first trimester abortions.
No history of alcohol, tobacco, or illicit drug use. Meds include ASA (started during
this pregnancy by PCP) and prenatal vitamins. Physical exam revealed new bilateral
pretibial bruises and multiple bruises in different stages of healing on legs and
arms. Labs revealed a normal CMP, CBC, PT 13 (INR 0.96), aPTT 34, negative ASO &nANA
titer, monospot, RPR, HIV, hepatitis panel. Fibrinogen was elevated at 509, D-dimer
0.6. Ristocetin cofactor, Factor VIII, beta-2-glycoprotein and hexagonal antibodies
were normal. All vWF multimers were present and normal. Mixing study and dilute Russell
Venom Viper Test (dRVVT) were abnormal confirming the presence of a lupus anticoagulant.
DISCUSSION
Thrombophilic disorders can be hereditary, acquired, or both. The antiphospholipid
antibody syndrome (APAS) is an acquired disorder associated with arterial and venous
thrombosis, recurrent miscarriages and thrombocytopenia due to the presence of anticardiolipin
antibodies, lupus anticoagulant, or subgroup of other antibodies. Types of thromboses
associated with the APAS are categorized into syndromes with specific treatment. Mixing
study and the dRVVT are specific tests to evaluate presence of a lupus anticoagulant.
As commonly seen, our patient's platelet aggregation disorder (vWF def) corrected
during pregnancy. Her history of spontaneous abortions prompted an APAS workup.The
presence of lupus anticoagulant confirmed our patient had the Fetal Wastage variant
of the APAS. Treatment for this is aspirin immediately before conception and heparin
immediately after continued till 6 weeks postpartum. In our patient Aspirin exacerbated
her vWF def causing bruising. We anticipate that the vWF def will resurface in the
postpartum period which together with treatment will increase bleeding. In an attempt
to prevent another abortion Aspirin was continued and heparin was added after discussion
of the risk and benefits of receiving anticoagulation and antiplatelet therapy with
the patient. A team approach by a high risk obstetrician, internist and hematologist
for close monitoring during and after pregnancy will be utilized.
DIARRHEA GIVES ME A HEADACHE: SAGITTAL VENOUS THROMBOSIS AS A COMPLICATION OF AN EXACERBATION
OF INFLAMMATORY BOWEL DISEASE
S. Homsi
1; J. Hefner1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116222)
LEARNING OBJECTIVES
1.) Recognize an uncommon extraintestinal manifestation of Inflammatory Bowel Disease
(IBD); 2.) Diagnose and treat a sagittal vein thrombosis; 3.) Recognize IBD as a hypercoagulable
state.
CASE
A 39-year-old woman, recently diagnosed with IBD, was admitted with severe watery,
bloody diarrhea associated with crampy abdominal pain, weight loss, and night sweats.
The patient was diagnosed six months prior to admission and never had a complete remission
of her symptoms. She was treated on this admission with solu-medrol (methylprednisolone),
pentasa (5-ASA) and rowasa (5-ASA) enema. On the fourth hospitalization day, the patient
developed a frontal headache, described as pressure-like radiating to the back of
her neck. The severity of the headache was initially 5/10, and increased significantly
over the next 24 hours to become 10/10. The headache worsened in the supine position
and improved mildly with sitting. There was associated nausea, vomiting and severe
photophobia without any focal neurological symptoms. She was initially treated with
NSAID's without any improvement. A non-contrasted head CT was negative for SAH or
a hemorrhagic CVA. Her GI symptoms continued despite IV steroids. 6MP was added without
improvement and then started on Infliximab. Physical examination revealed no nucal
rigidity and no focal neurologic deficits. Fundoscopic exam revealed no papillary
edema. Brain MRI revealed a thrombus involving the superior sagittal sinus extending
into the left transverse sinus. A hypercoagulable work up revealed decreased levels
of Protein S. The patient was started on a heparin drip and the headache improved
dramatically over the next 48 hours. She was discharged on warfarin.
DISCUSSION
The incidence of extraintestinal manifestations of inflammatory bowel disease is reported
to be between 25 and 35%. Neurological complications are rare and are usually the
result of thromboembolic events. The pathophysiology is not fully understood but there
may be an increased frequency of thrombophilic mutations such as factor V Leiden,
prothrombin, or methylene tetrahydrofolate reductase. The coagulation abnormalities
that have been reported to be associated with Crohn's disease include accelerated
thromboplastin generation, increased concentration of factor V, factor VIII, and fibrinogen.
Low antithrombin III concentrations, thrombocytosis, decreased platelet survival,
and spontaneous platelet aggregation causing coagulation abnormalities have also been
reported. The most common presentation of intracranial venous thrombosis is headache
associated with symptoms of increased intracranial pressure. MRI is the gold standard
for diagnosis and treatment consists of anticoagulation.
DIC AND TTP: TWO, THREE LETTER WORDS
M. Hamblin
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117472)
LEARNING OBJECTIVES
1. Appreciate the overlapping clinical and laboratory findings in DIC and TTP. 2.
Recall the pathogenesis behind these two disorders.
CASE
A 45-year-old man with HIV presented with ten days of headaches. His examination was
normal with no signs of meningismus. Cerebrospinal fluid was positive for cryptococcal
antigen, and he was treated with intravenous amphotericin. On the third day he developed
an acute change in his mental status thought to be due to sepsis. His altered mental
status persisted over the ensuing seven days despite antibiotics. He developed a fever
and his creatinine increased from 1.5 to 3.0; his platelet count decreased from 164
to 20. His fibrinogen and PTT remained in the normal range, but the D-dimer was positive.
His PT was 16.7, and his LDH was 1,124. A blood smear revealed schistocytes indicating
microangiopathic hemolytic anemia.
DISCUSSION
Thrombotic thrombocytopenic purpura (TTP) and disseminated intravascular coagulation
(DIC) have overlapping lab features that can make a definitive diagnosis difficult.
TTP is due to an acquired antibody-mediated deficiency of von Willebrand factor (vWF)-cleaving
protease. It is thought that antigenic mimicry from an antecedent infection stimulates
the development of this antibody. In HIV patients a link between CMV infection and
the development of TTP has been established. The decreased levels of vWF-cleaving
protease lead to intravascular fibrils that induce the thrombocytopenia and microangiopathic
hemolytic anemia with a markedly elevated LDH. The clinical symptoms of fever, neurologic
changes, and renal insufficiency result from obstructed small-vessel blood flow. The
neurological findings are often the first clinical indication of disease, and may
range from headache and confusion to aphasia, lethargy, and coma. In DIC the depletion
of inhibitors of coagulation activate thrombin. The coagulation cascade becomes unregulated
resulting in the hallmark lab findings of DIC: thrombocytopenia, hypofibrinogenemia,
increased fibrin degradation products, and a prolonged prothrombin time (PT). In twenty-five
percent of cases, microangiopathic hemolytic anemia may be present. Distinguishing
between these two diseases is important as they warrant different treatments. In DIC,
treating the underlying disease process is essential. Supportive measures, such as
transfusions with platelets and cryoprecipitate maintain coagulation factors necessary
to prevent bleeding until the underlying disease is eradicated. Low-dose heparin may
prevent errant thrombosis. In the case of TTP, treatment is aimed at removing the
antibody to von-Willebrand factor cleaving protease using large volume plasmapheresis.
DIFFUSE ALVEOLAR HEMORRHAGE, PULMONARY CAPILLARITIS AND RENAL FAILURE: A CASE OF CATASTROPHIC
ANTIPHOSPHOLIPID SYNDROME
B.C. Clark
1. 1University of California, San Francisco, San Francisco, CA. (Tracking ID #116421)
LEARNING OBJECTIVES
1) Recognize the major clinical manifestations of catastrophic antiphospholipid syndrome.
2) Review treatment strategies for catastrophic APLS.
CASE
A 42 year old woman with a remote history of infective endocarditis and mitral valve
replacement was transferred from an outside hospital for work-up of hemoptysis and
pulmonary hypertension. She reported 8 months of daily expectorated blood clots and
blood streaked sputum as well as marked progressive dyspnea on exertion. Review of
old records revealed long-standing mild thrombocytopenia, elevated PTT, false positive
RPR and strongly positive anticardiolipin (aCL) IgG anti-body. On exam, lungs were
clear. Laboratory data revealed a platelet count of 114,000, PTT of 62, and creatinine
of 2.0 mg/dL. Urinalysis was bland. Anticardiolipin IgG was markedly elevated at 122
GPL, and Russell viper venom test was elevated to 112. ANA was borderline positive
at 1:40 but DS DNA and anti-Smith were both negative, and complement levels were normal.
ANCA and glomerular basement membrane anti-bodies were also negative. A chest radiograph
showed interstitial infiltrates and a high-resolution CT scan showed diffuse ground
glass opacities. Broncho-alveolar lavage revealed hemosiderin laden macrophages with
no evidence of infection. A mini-thoracotomy with lung biopsy revealed evidence of
hemorrhage with capillaritis. Shortly after the procedure the patient's clinical condition
worsened further, with development of acute renal failure despite aggressive hydration.
Creatinine rose to 3.8 mg/dL and pulmonary function deteriorated. The patient was
treated with high dose solumedrol and underwent plasmapheresis three times. Her clinical
condition rapidly improved, pulmonary hemorrhage cleared, creatinine fell to 2.3 mg/dL
and aCL antibody declined to 35. Shortly after she underwent renal biopsy which revealed
microthrombi of the arterioles consistent with renal involvement in antiphospholipid
syndrome.
DISCUSSION
The antiphospholipid syndrome is defined by venous or arterial thromboses, recurrent
fetal loss and the presence of elevated levels of antiphospholipid antibodies. A subset
of patients with APLS develop a “catastrophic” clinical picture characterized by vaso-occlusive
events, typically microthromboses, affecting multiple organs over a period of days
to weeks. Diverse organs, including the heart, lungs, kidneys, liver, spleen, CNS
and extremities can be involved. In the largest case series of 80 patients, renal
involvement occurred in 72% and pulmonary involvement in 64% of cases. Lung manifestations
can include pulmonary emboli, pulmonary hypertension, ARDS, and intra-alveolar hemorrhage
and capillaritis as in this patient. Thrombotic microangiopathy is the most common
renal manifestation. Laboratory findings include elevated titers of aCL (98%), lupus
anticoagulant (68%), and thrombocytopenia (60%). Treatment modalities include anticoagulation,
high-dose steroids, plasmapheresis, IV gamma globulin, and immunosuppression with
cyclosporine or azathioprine. In a 1998 case series of 50 patients, treatment with
a combination of anticoagulation, steroids and plasmapheresis or IV GG resulted in
a 70% survival rate. The mainstay of therapy, anticoagulation, was delayed in this
patient until she was safely past the immediate post-operative period and pulmonary
hemorrhage had subsided.
DIFFUSE LYMPHADENOPATHY IN A PATIENT WITH LONG-STANDING HIV
K. Ozer
1; J. Madrazo1; L. Lu1. 1Baylor College of Medicine, Houston, TX. (Tracking ID #116772)
LEARNING OBJECTIVES
1. Construct the differential diagnosis of diffuse lymphadenopathy. 2. Review the
clinical presentation and histopathologic features of Castleman's Disease. 3. Recognize
intra-abdominal lymphadenopathy as a cause of biliary obstruction
CASE
A 34 year-old man with an eleven-year history of HIV presented with a 3-week history
of right upper quadrant pain, fever, and 10 lb weight loss. The pain was described
as a constant, stabbing pain with radiation to the back and was not associated with
food intake. Physical examination revealed a temperature of 98.7°, mild scleral icterus;
bilateral rubbery, mobile, non-tender submandibular, cervical and axillary lymphadenopathy;
tender right upper quadrant, with no rebound tenderness or guarding. Hepatosplenomegaly
was not present. Labs revealed CD4 count of 105, viral load greater than 750,000,
WBC: 3.4, Hb: 9.4, Hct: 27.4, MCV: 86.9, PLT: 158, Total bilirubin: 3.1, AST: 90,
ALT: 122, alkaline phosphatase: 666. CT of the abdomen showed extensive retroperitoneal
lymphadenopathy, including a node on the gastrohepatic ligament compressing the biliary
tree. Work up for Bartonella, mycobacterial, fungal, CMV and EBV infections were negative.
The patient had an excisional biopsy of a cervical lymph node, which was consistent
with the plasma cell variant of Castleman's Disease (CD). The patient was treated
with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) and responded well
with rapid improvement of the biliary obstructive symptoms.
DISCUSSION
Diffuse lymphadenopathy is associated with a wide range of conditions including infections,
immunologic diseases, malignancies, and other diseases of lymphoid tissues such as
histiocytic necrotizing lymphadenitis, lymphomatoid granulomatosis, dermatopathic
lymphadenitis and Castleman's Disease. Castleman's Disease is a rare entity and its
prevalence has not been well documented. It can present in two forms, localized form
(unicentric) and diffuse form (multicentric). Unicentric CD is found in non-HIV patients.
Multicentric CD is often associated with HIV infection, especially with co-infection
of Human Herpesvirus 8 (HHV8). CD is prevalent in young males who typically present
with vague constitutional symptoms of weight loss, fever, malaise, and diffuse lymphadenopathy.
Lymph node biopsy is required for definitive diagnosis. Treatment options include
combination chemotherapy with several regimens, the most commonly used being CHOP.
Unicentric CD is curable with surgical resection. Multicentric CD has poor prognosis
with overall mortality of 70–85% and a mean median survival of 8 to 14 months. In
conclusion, multicentric CD should be on the differential diagnosis in HIV patients
presenting with diffuse lymphadenopathy.
DISSEMINATED COCCIDIOIDOMYCOSIS
P. Hu
1; M. Pillai1; N.C. Le1. 1Baylor College of Medicine, Houston, TX. (Tracking ID #116476)
LEARNING OBJECTIVES
1) Recognize the presentation and diagnosis of disseminated coccidioidomycosis 2)
Treatment for coccidioidomycosis
CASE
A 49-year-old African American man with a past medical history of hepatitis C, cirrhosis,
and frequent travel to Arizona, presented with a two-week history of fever, chills,
and cough. Significant findings on admission were temperature 101.3, RR 28 and decreased
breath sounds on the left base. Lab results showed WBC 15K, with 85% neutrophils and
2% bands. Chest x-ray showed a left pleural effusion. Various antibiotics were started
without improvement. On day #9, sputum culture grew a fungal pathogen, which was later
identified as
Coccidioides immitis
. On day #10, patient developed several subcutaneous nodules in his forearms. Biopsy
of the nodules showed
C. immitis
. He is currently on fluconazole 400 mg daily with resolution of symptoms.
DISCUSSION
C. immitis
is a soil saprophyte endemic to California, Arizona, and Western Texas. Primary pulmonary
disease usually goes unnoticed. Patients can present with a range of symptoms from
cough to severe pneumonia. The disease is usually self-limited, but two important
sequelae can occur: 1) hypersensitivity reactions and 2) dissemination. In hypersensitivity,
the patient can develop conditions such as erythema nodosum, erythema multiforme.
Dissemination occurs primarily with hematogeneous spread and is more likely in African
Americans, pregnant women, and immunosuppressed patients. Diagnosing coccidioidomycosis
involves recovery of
C. immitis
from clinical specimens or detection of anti-coccidioidal antibodies in serum or other
body fluids. Recovery of C. immitis from respiratory secretions, tissue and other
specimens establishes the diagnosis since it is not a normal flora. Anti-coccidioidal
antibodies are highly specific (80–90% sensitivity in disseminated disease), but these
antibodies may lag behind the onset of illness from weeks to even months. Thus, the
absence of the antibodies does not exclude the diagnosis of coccidioidomycosis. Treatment
depends on the severity of the disease. Primary pulmonary disease usually resolves
without treatment. For disseminated disease, oral azole antifungal agents are the
drugs of choice. Amphotericin B is used in patients with severe disease (eg. spread
to the spine) and during pregnancy. Treatment with antifungal should be continued
for years. Prognosis for complete cure remains uncertain.
DOC, I COUGHED UP A WORM!
M. Traina
1; R.R. Cader2. 1University of California, Los Angeles—San Fernando Valley Program,
Sylmar, CA; 2University of California, Los Angeles, North Hills, CA. (Tracking ID
#117536)
LEARNING OBJECTIVES
1) To learn the clinical presentation of anisakis infection. 2) To learn management
of anisakis infection.
CASE
A 56 year old male presented to urgent care complaining of coughing up a worm. The
patient killed the worm and kept it in a wrapper. He denied fevers, chills, diarrhea
or abdominal discomfort. Upon further review, he noted that he had been consuming
raw beef for the past few months. One week prior to presentation, he had started eating
raw fish including salmon and mackerel purchased at the Santa Monica Fish Market.
Physical examination was normal with no evidence of abdominal distention or tenderness.
The worm was taken for analysis and identified to be Anisakis simplex.
DISCUSSION
Anisakiasis is caused by infection with the nematode Anisakis simplex. A. Simplex
is found in whales, dolphins, seals and sea lions. Eggs are ingested by tiny crustaceans
which are then ingested by fish, where Anisakis matures into larvae. Humans are accidental
hosts upon ingestion of raw parasitized fish and crustaceans. In the US, infection
occurs most commonly with ingestion of salmon, mackerel, herring and cod. Patients
usually present with an itchy sensation in the back of the throat after ingestion,
followed by coughing up the worm as was seen with our patient. If the parasite is
swallowed, Anisakis can be invasive causing a severe eosinophilic granulomatous response
usually 1–2 weeks after ingestion. Patients can present with abdominal distention
or obstruction and present with symptoms that mimic appendicits or crohn's disease.
Spontaneous regurgitation or endoscopic removal of worm cures infection. Occasionally,
surgical debridement may be needed for invasive disease. Diagnosis is made by removal
of larvae with subsequent microscopic analysis. There is no known antiparasitic therapy
for anisakiasis. Infection can be prevented by cooking to >60°C or freezing to lower
than −20°C for at least 48 hours. It is important to educate patients as to the risks
of eating improperly prepared foods. Our patient was advised to discontinue eating
raw meats and fish.
DON'T EAT AT JOES
M. Cordone
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117407)
LEARNING OBJECTIVES
1. Recognize the presenting features of Hepatitis A. 2. Diagnose and manage patients
with Hepatitis A 3. Repeat labs if there remains a strong clinical suggestion of a
common etiology.
CASE
A 29 year-old woman presented with two weeks of nausea, fever and vomiting. She reported
having a hamburger at a Cajan barbecue less than two weeks ago. She worked in a veterinary
hospital where she incurred numerous animal scratches and bites. She was tachycardic,
hypotensive, and had tenderness of the right upper quadrant. She had 23% bands, but
a normal WBC. Her amylase was 152; she had a negative pregnancy test, and an AST and
ALT of 158 and148. Her alkaline phosphatase and total bilirubin were normal. Her TSH
was 0.24. Fearing thyroid storm, the emergency physician administered PTU and propranolol.
Her transaminases rose to AST 3,281 and ALT of 3,265 over the next day. A CT scan
revealed peri-portal edema secondary to hepatitis. A viral hepatitis panel was normal.
Her toxicology screen, including aspirin and acetominophen were normal. ANA, CMV,
and a Monospot test were negative; the ferritin was 6,018. Owing to a high clinical
suspicion, a second hepatitis panel was ordered. The second hepatitis panel was reactive
to IgM Hep A antigen.
DISCUSSION
The incubation period of hepatitis A is four weeks. It is transmitted via a fecal-oral
route. Clinical symptoms include nausea, anorexia, vomiting, fatigue, malaise, headache,
fever, pharyngitis, cough, and myalgias. Aminotransferases have a variable rise from
400 to 4,000, but do not correlate with liver damage. Neutropenia and lymphocytopenia
are also present with some atypical lymphocytes, making it difficult to distinguish
from infectious mononucleosis. The high clinical suspicion in this case prompted repeating
a study that made sense in the first place, thereby obviating more invasive surgical
and biopsy procedures. Physicians should be aware of the incubation period of Hepatitis
A. This case illustrates an important second lesson: when a clinical case is confusing,
it is always better to repeat tests that made sense in the first place then to embark
on invasive and fantastical work-ups.
EFFUSION CONFUSION
K. Casey
1; J. Newman1; J.M. Huddleston1. 1Mayo Clinic, Rochester, MN. (Tracking ID #117052)
LEARNING OBJECTIVES
To recognize pulmonary amyloid as a cause for persistent non-cardiac pleural effusions.
CASE
An 84-year-old male presented with persistent pleural effusions. The patient was evaluated
three months prior for shortness of breath. His only history was hypertension and
a bypass surgery six years prior. Chest x-ray revealed a left-sided pleural effusion.
The patient was treated for presumptive congestive heart failure and discharged on
furosemide and an ACE-I with moderate improvement. His dyspnea persisted for three
months and he was readmitted with a diagnosis of pneumonia. After a course of levofloxacin,
the patient was discharged from the hospital. He returned one week later with worsening
dyspnea, cough and hemoptysis. Chest x-ray revealed a slightly worse pleural effusion.
A 2D-echo showed normal left ventricular function. Ultrasound-guided thoracentesis
yielded one liter of slightly hemorrhagic fluid. The fluid was negative for organisms,
acid fast bacilli, and malignancy. CT scan and bronchoscopy were also not diagnostic.
Subsequently, the patient underwent wedge resection of the left upper lobe for definitive
diagnosis. A chest tube drained sero-sanguinous fluid. He was transferred to the Mayo
Clinic for further treatment of the effusion. Pathology review of the lung biopsy
at the Mayo Clinic revealed alveolar septal amyloidosis.
DISCUSSION
Amyloidosis refers to a group of conditions characterized by the deposition of abnormal
protein material in extracellular tissue. Major sites of clinically significant amyloid
deposition are in the kidneys, heart and liver. Primary amyloid (AL) is due to deposition
of protein derived from immunoglobulin light chain fragments. Though pulmonary involvement
with primary systemic amyloidosis has been shown in various post-mortem autopsy reports,
cases presenting with pulmonary amyloid have been reported infrequently. Because cardiac
involvement in systemic amyloid is common, it is often difficult to discern the origin
of pleural effusions. Our patient was noted to have hemorrhagic effusions and no evidence
of heart failure by echo. This suggests that the etiology of the effusions was pulmonary
rather than cardiac. In our patient, fat biopsy confirmed the diagnosis of systemic
amyloid and bone marrow biopsy revealed 5% monoclonal lambda plasma cells. The effusions
subsequently improved and the chest tube was removed. The patient was discharged in
stable condition and shortly after was started on melphalan and prednisone for the
treatment of systemic amyloid. He is presently doing well.
ELEVEN ISN'T ENOUGH: FACTOR XI DEFICIENCY AND CORONARY ARTERY DISEASE
F. Yafai
1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116261)
LEARNING OBJECTIVES
1. Recognize the clinical manifestations of factor XI deficiency. 2. Recognize that
factor XI deficiency does not protect against myocardial infarction (MI). 3. Recognize
the importance of risk benefit analysis in starting aspirin in patients with factor
XI deficiency and coronary disease (CAD).
CASE
An 85 year old Caucasian male physician was seen following 3-vessel coronary bypass
after a myocardial infarction. Twenty years prior, the patient was diagnosed with
factor XI deficiency after an incidental finding of an elevated PTT. He denied history
of epistaxis, melena, hematochezia, but endorsed easy bruising and occasional bleeding
with teeth-brushing. He denied history of spontaneous bleeding, but reported increased
bleeding after an inguinal hernia repair, dermatologic procedure and an attempted
steroid injection. He had an uncomplicated cholecystectomy, dental extractions and
colonoscopies. During the bypass, he received fresh frozen plasma. On exam, there
were several ecchymoses along his upper extremities but no signs of active bleeding.
His hospital course was uneventful, without excess bleeding. Admission labs revealed
PTT 66.1, (baseline 60–80). Post-operative (post fresh frozen plasma) labs revealed
PTT 32.4, PT 12.2 INR 1.1, Hgb 9.6 and platelets 131. The surgeons requested consultant
input on aspirin administration to prevent graft restenosis. After lengthy discussions
between hematology, cardiology and cardiothoracic surgery, it was decided that the
patient should be discharged on a low dose aspirin. The decision was based on a review
of the literature and a review of the patient's history.
DISCUSSION
Factor XI deficiency, previously known as hemophilia C, is diagnosed by an isolated
elevation in PTT. It tends to present with milder bleeding episodes than other coagulopathies.
In addition, it has not been shown to be protective against myocardial infarction.
Studies of patients with hemophilia A and B have demonstrated 80% reduced risk of
myocardial infarction. There is 25% reduced risk among female carriers of hemophilia.
In contrast, a recent study of 96 Israelis with factor XI deficiency found that the
incidence of acute myocardial infarction was similar to that of the general Israeli
population. Furthermore, anecdotal reports of patients with factor XI deficiency and
coronary disease have demonstrated that aspirin is well tolerated. However, more research
is required. Until then, risk/benefit ratios should be defined for each individual.
Our patient had significant coronary disease, recent bypass and no serious bleeding
episodes. Based on risk benefit analysis and physician-patient discussion, it was
decided to initiate therapy with low dose aspirin.
EPHEDRINE STRIKES AGAIN? A CASE REPORT AND LITERATURE REVIEW
E. Mcdonald
1; J.I. Lane1. 1Mayo Clinic, Rochester, MN. (Tracking ID #116553)
LEARNING OBJECTIVES
Recognize uncommon causes for stroke in young adults.
CASE
A 36-year-old woman presented to the Emergency Department (ED) with headache, dizziness,
nausea, vomiting, and abdominal discomfort. She had already been seen at an outside
ED and was given a course of ciprofloxacin and prochlorperazine, which she had completed.
A migraine headache was diagnosed and she was sent out with promethazine. She returned
to the ED three days later, still complaining of headache. She was referred to an
outpatient resident's clinic without ED evaluation. The resident noted hypokalemia
in the second ED evaluation and admitted the patient to the hospital for rehydration.
On admission, her family stated that she had been extremely lethargic the past few
days, only getting out of bed to go to the bathroom, with assistance. A non-contrast
CT of the head showed an hemispheric ischemic infarct in two-thirds of the right cerebellum
with associated mass effect. A diffusion weighted MRI and MRA of the head the next
morning showed a subacute infarct in the right posterior inferior cerebellar artery
(PICA) with compression of the fourth ventricle and dilation of the third and lateral
ventricles. A cerebral angiogram showed occlusion of the right PICA and evidence for
bilateral vertebral artery dissections with no evidence of fibromuscular dysplasia
(images, including 3D, presented in poster). An extensive thrombophilia work-up did
not reveal any coagulation disorder. The patient had no risk factors for or family
history of stroke. She was started on anticoagulation and showed no further signs
of cerebral edema. A urine drug screen was done shortly after admission, which confirmed
the presence of phenylpropanolamine (PPA) and ephedrine. After questioning, the patient's
husband brought in a bottle of “diet” pills that had ephedrine listed as one of the
ingredients. It was unclear whether the patient had taken these pills recently and/or
an over-the-counter cough and cold medicine.
DISCUSSION
PPA used to be commonly found in appetite suppressants and in some cough and cold
medicines. It was slated to be taken off the market after an FDA advisory in November
of 2000. It is associated with hypertension (Cantu, et al., 2003) and is an independent
hemorrhagic stroke risk factor in women (Kernan, et al., 2000). Dietary supplements
with ephedra alkaloids (ma huang) are also associated with cardiovascular and central
nervous system adverse events (Haller and Benowitz, 2000). The FDA announced plans
to ban the sale of ephedrine on December 30th, 2003. Scientific evidence for this
action is reviewed in the poster. PPA and ephedrine ingestion with resulting hypertension
and subsequent vertebral artery dissection may be the mechanism of massive stroke
in this otherwise healthy young woman.
EXTRA ADRENAL PARAGANGLINOMA: SYMPTOMS IT MAY CAUSE
T.M. Feinstein
1; T. Bui1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115446)
LEARNING OBJECTIVES
1. Recognize pheochromocytoma as a cause of glucose intolerance and ischemic bowel.
2. Diagnose pheochromocytoma using clinical, biochemical and radiological indicators.
CASE
71 year old Caucasian female with a history of COPD, bronchiectasis, myelodysplastic
syndrome, recently diagnosed diabetes and worsening anxiety presented with left lower
quadrant abdominal pain, headache, excessive perspiration, orthostatic hypotension
and elevated WBC. Abdominal CT and ultrasound revealed a sigmoid mass (6 × 5 cm) with
central necrosis, separated from the uterus and ovaries, with vascularity within the
sigmoid mesocolon. Colonoscopy showed ischemic colitis. Her blood pressure became
labile and fluctuated from 200/100 to 80/50 in minutes. She experienced momentary
mental status changes, nausea and constipation. EKG and cardiac enzymes show no evidence
of a myocardial infarction. 24 hour urine catecholamines and serum catecholamines
were significantly elevated. I-131 metaiodobenzylguanidine (MIBG) scintigraphy showed
increased uptake in her left pelvic mass, without other foci. Phenoxybenzamine and
clonidine controlled blood pressure. Nitroprusside was used during the open, uncomplicated
laporatomy. Pathology revealed a 70.7 gram extra adrenal paraganglioma. Urine catecholamines
normalized. The patient's symptoms improved and her blood pressure was controlled
with a low dose of metoprolol.
DISCUSSION
Pheochromocytoma is a catecholamine-secreting tumor. Common manifestations are headache,
excessive perspiration and palpitations. Altered mental status, focal neurological
signs, seizures, or stroke may be observed during a paroxysm. Unusual presentations
include diabetes mellitus and gastrointestinal complications. Glucose intolerance
is present in one-third of patients. Decreased insulin sensitivity and secretion cause
carbohydrate intolerance. Pheochromocytoma has an atypical relationship with type
2 diabetes. Diabetic symptoms worsen as the patient's body mass index decreases from
catecholamine-induced lipolysis. Glucose intolerance associated with normal body weight
and hypertension is a clue in the diagnosis. Gastrointestinal ischemic complications
are rare but are more frequent with larger tumors (>70 grams). Biochemical markers
are usually elevated 3 times normal. Urine normetanephrine is 97% sensitive. Norepinephrine
and epinephrine or normetanephrine and metanephrine are 100% sensitivity when combined.
Hydroxymethoxymandelic acid has a sensitivity of 70% with the 95% confidence level
(48 mg/g). Plasma catecholamines are more useful when urine values are equivocal.
The MIBG scan reveals uptakes by the pheochromocytoma, and it can localize tumors
with a sensitivity of 85% and a specificity of 99%.
FALANGA: AN UNEXPECTED CAUSE OF PERIPHERAL NEUROPATHY
E.J. Rourke
1; S.S. Crosby1; M. Paasche-Orlow1; M.A. Grodin1. 1Boston University, Boston, MA.
(Tracking ID #116708)
LEARNING OBJECTIVES
1. Diagnose history of falanga as a cause of peripheral neuropathy 2. Recognize the
possibility of torture as an etiology for otherwise unexplained symptoms in patients
from high-risk areas.
CASE
A 38-year-old male from Mauritania with a history of chronic hepatitis B, latent TB
infection treated with isoniazid for nine months, depression being treated with mirtazapine,
and post traumatic stress disorder presented to Primary Care complaining of longstanding
bilateral foot pain. The pain was described as severe burning, associated with numbness,
which radiated into the midcalf. The patient described the sensation as “stones in
the bottom of his feet,” and stated that the pain was most noticeable at night, preventing
him from sleeping. Physical exam revealed decreased sensation below the mid-shin to
vibration, light touch, and cold, with minimal pain to palpation on the soles of the
feet bilaterally, no visible deformity, and normal deep tendon reflexes. The rest
of his exam was normal. CBC, chemistries, liver function tests, hemoglobin A1C, TSH,
B12, folate, RPR, cryoglobulins, SPEP, UPEP, and ANA were all normal. HIV 1 and 2,
hepatitis C, and hepatitis Be serologies were negative, although hepatitis B antigen
was positive. EMG testing revealed moderate to severe peripheral neuropathy, primarily
demyelinating in nature, and the patient was diagnosed with sensory distal polyneuropathy,
possibly related to chronic hepatitis B infection. At this time, the patient revealed
a history of falanga while detained in Mauritania in 1989. The patient stated that
he had been suspended by his wrists and ankles from a horizontal pole, and beaten
on the soles of his feet repeatedly with a baton, causing severe swelling and pain
that prevented him from walking for three weeks. MRI of the feet revealed increased
signal within the plantar interosseous and lumbrical muscles bilaterally, consistent
with muscle injury or inflammation. The patient was treated with amitriptyline and
a lidocaine patch, producing partial relief of his symptoms, and also continued therapy
for depression and post traumatic stress disorder.
DISCUSSION
Amnesty International documents the practice of torture in more than 150 different
countries, including 30 countries in which falanga is practiced. There may be over
500,000 survivors of torture residing in the United States. Torture survivors seek
attention in primary care settings for medical and psychological consequences of torture,
and may have difficulty disclosing or describing their experiences due to traumatization.
Physicians should be aware of the possibility of a torture history in patients from
high-risk areas. Comprehensive information about the diagnosis and treatment of torture
survivors is available in the Istanbul Protocol. []
FATAL MIGRAINOUS STROKE IN YOUNG MALE
S. Habis
1; L. Cation1. 1University of Illinois at Peoria, Peoria, IL. (Tracking ID #115846)
LEARNING OBJECTIVES
To recognize the typical and atypical features and presentations of stroke as complication
of migraine.
CASE
A 37 year old male with a history of migraine with aura (IHS criteria) was found unresponsive
in bed. He was on no medication, and there was no history of smoking, Alcohol or illicit
drug abuse. Upon arrival to ER, he was intubated. Vital signs were stable and neurological
exam showed Glasgow coma scale 6, PERRL, positive gag and corneal reflexes, dense
right hemiplegia, spontaneous left side movement, symmetric DTR's with downgoing toes.
The rest of the exam was normal. The initial evaluation showed normal CBC, chemistries,
coagulation profile and negative toxicology screen. A head CT scan (Figure 1) showed
acute ischemic infarct in the ACA and MCA territories. Further evaluation including
echocardiogram, CXR, ESR, and hypercoagulable state profile were normal. Brain MRI
showed acute ischemic infarct in the MCA and distal ACA territories. MRA was normal
(Figure 2). The patient was stable on the second day, but Glasgow coma scale was 3
on the third and fourth day and he died on the fifth day. The brain autopsy (Figure
3) showed cerebral ischemia (ACA and MCA) and edema with evidence of subfalcial and
uncal herniation on the left —despite maximal medical management—, and new hemorrhagic
infarct in the left PCA territory due to compression of the PCA by hippocampal and
parahippocampal notching. There was no evidence of atherosclerosis, vasculopathy or
embolic event. The final diagnosis was migrainous stroke.
DISCUSSION
Migrainous stroke reportedly causes 1.4% of all strokes in young adults with female
gender predominance (statistical differences were noticed in the few studies found
in the literature). The posterior circulation especially the PCA territory is the
more frequently involved area in migrainous stroke and the size of the infarct is
generally small. Fatality in acute stroke, regardless of etiology, range between 1.5–7.3%.
Review of literature regarding migraine and stroke between 1977 and 1997 showed that
only 44 of 500 patient reported presented with ischemic stroke occurred during acute
migraine and that more cases occurred with migraine without aura than with aura. This
case is particularly unusual because it affected a male, in anterior circulation territory,
and had fatal outcome. We were unable to find a case of fatal migrainous stroke in
a young male previously reported in the literature. In summary, we report a case of
migraine associated fatal stroke.
FEVER AND CERVICAL LYMPHADENOPATHY
N.S. Shah
1; P. Bhat1. 1Columbia University, New York, NY. (Tracking ID #115419)
LEARNING OBJECTIVES
1) Discuss the differential diagnosis of cervical lymphadenopathy and fever in a young,
healthy patient, and 2) Identify Kikuchi-Fujimoto disease as a rare, benign cause
of cervical lympadenopathy.
CASE
A 24 year-old healthy female presented with 1 month of fevers and unilateral cervical
lymphadenopathy. Two-weeks prior to admission she completed a course of broad-spectrum
antibiotics without clinical improvement. The patient immigrated from Trinidad 6 years
prior and had no history of recent travel, trauma, or contact with animals. She is
married, and does not smoke or drink alcohol. She was PPD-negative within the past
year. Review of systems was notable for absence of weight loss, dysphagia, or rash.
Exam revealed right cervical fullness with multiple soft, mobile, tender lymph nodes,
and no erythema or fluctuance. Initial leukocyte count was 5800/L with 81% neutrophils;
basic chemistries, liver enzymes, and rheumatologic screen were within normal limits.
Sedimentation rate was markedly elevated at 131 mm/hr. Blood and urine cultures were
negative. PPD was again negative during the admission. Neck CT revealed multiple,
large 1.5–2.0 cm, right upper neck nodes with heterogeneous enhancement, and extensive
subcutaneous edema. Fine needle aspiration (FNA) and core biopsy were non-diagnostic,
so excisional biopsy was pursued. Histopathology showed near-total effacement of lymph
node architecture and replacement by histiocytes, apoptotic cells and nuclear debris
consistent with Kikuchi-Fujimoto disease. Special stains for fungi, bacteria, acid-fast
bacilli, Epstein-Barr virus, and spirochetes were negative. No atypical cells consistent
with malignancy were found. At evaluation two months after hospital discharge the
patient reported complete resolution of symptoms.
DISCUSSION
The differential diagnosis of cervical lymphadenopathy with fever is extensive, and
includes a variety of infectious causes such as cat scratch disease, mononucleosis,
mycobacterial infections, bacterial adenitis; head and neck cancers such as Hodgkin's
and non-Hodgkin's lymphoma; and other causes such as Kikuchi-Fujimoto disease. Careful
history provides important diagnostic clues, so patients should be probed for exposures,
travel, high-risk behaviors, and constitutional symptoms. Definitive diagnosis is
made by culture and histology. FNA may be useful in cases of infection, however excisional
biopsy provides greater detail about lymph node architecture for diagnosis of malignancy.
Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis) is a rare, benign
condition of unknown etiology characterized by cervical lymphadenopathy and fevers
seen predominantly in young women. Initially described in Asians, the disease is now
reported in persons of all races. No effective treatment has been established, but
the majority of cases are self-limited within 2–3 months.
FEVER OF UNKNOWN ORIGIN ASSOCIATED WITH LABILE HYPERTENSION
Y.Y. Li
1; J. Hefner1; R. Granieri1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking
ID #115952)
LEARNING OBJECTIVES
(1) Recognize the differential diagnosis of fever of unknown origin (FUO) (2) Recognize
the unusual presentations of pheochromocytoma (3) Manage hypertension in patients
with pheochromocytoma.
CASE
An 83 year-old female with history of valvular heart disease was admitted to the hospital
with a 2 month history of fever, sweats and labile hypertension resistant to therapy
with levofloxacin and amoxicillin/clavulanate. She complained of headache, dizziness
and dyspnea on exertion. She denied cough, chest pain, nausea, vomiting, abdominal
pain, or dysuria. On admission the patient developed rapid atrial fibrillation. Physical
examination was remarkable for a temperature of 38.4 degree Celsius, BP 160/100 mmHg,
an irregularly irregular heart rhythm, a III/VI pansystolic murmur at the apex, and
lower extremity edema. Chest x-ray did not show infiltrate. She was started on ampicillin
and gentamicin for subacute bacterial endocarditis although multiple blood cultures
were negative and TEE failed to demonstrate definitive vegetations. When fevers persisted,
the antibiotics were changed to vancomycin and ceftazidime, again without benefit.
Antibiotics were discontinued. CT scans of the chest and abdomen showed congestive
failure with a left pleural effusion and a 5 × 5 cm left adrenal mass which was confirmed
to be pheochromocytoma by measurement of catecholamines and histopathology. She continued
to have fevers, tachyarrhythmias and labile hypertension despite therapy with alpha
and beta blockers. She was not thought to be a surgical candidate. The family elected
hospice care according to the patient's advance directives.
DISCUSSION
A precise diagnosis of FUO in the elderly can be made 87–95% of the time. Often FUO
in the elderly is the result of an atypical presentation of common diseases. Infection
is the etiology in 25–35%, connective tissue disease in 25–31%, and malignancy in
12–23% of the cases. After thorough history and physical, focusing on symptoms and
signs of intra-abdominal diseases, cardiac and musculoskeletal disorders, tuberculosis
and cancers, CXR and basic laboratory studies including ESR, imaging of the abdomen,
blood cultures and TEE should be done. All non-essential drugs should be discontinued.
Gallium-67 or indium-111 labeled leukocyte scanning may identify infection or malignancy.
The presentation of fever, labile hypertension and tachyarrhythmia in this patient
can be explained by the pheochromocytoma. Pheochromocytoma may present with fever
by producing the internal pyogen interleukin-6. The definitive treatment is removal
of the tumor after blood pressure is controlled with both alpha and beta blockers.
Beta blockers alone are contraindicated due to resultant unopposed alpha stimulation.
FOR PULMONARY EMBOLISM IS D-DIMER REALLY A SNOUT?
J. Kamali
1; M. Elnicki1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116489)
LEARNING OBJECTIVES
1) To recognize the importance of pre-test probability of disease in the interpretation
of diagnostic test results, 2) To recognize limitations in the use of the D-Dimer
test to rule out pulmonary embolism (PE).
CASE
A 46 year-old woman with a history of hypertension, type 2 diabetes mellitus and obesity
presented with a sudden onset of right-sided pleuritic chest pain of 10 hours duration.
The pain began at rest and was sharp, 8/10 in severity and nonradiating. There were
no alleviating factors. The patient denied any trauma, recent operations, fever or
chills but had a sedentary life style. She was nonsmoker and denied alcohol or illicit
drug use. No family history of coagulopathy. Vital signs were normal except respiration
rate of 22/minute. Pulse oximetry was 99% on room air. Heart, lung and extremities
were unremarkable, but there was moderate right chest wall tenderness on palpation.
Laboratory data were notable for normal electrolytes, cardiac enzymes and electrocardiogram.
D-Dimer test was negative and a V/Q Scan was read as low probability for PE. Lower
extremity dopplers were negative for deep venous thrombosis (DVT). Due to the continued
high clinical suspicion of a PE, a pulmonary angiogram was performed which demonstrated
filling defects in 3 segmental branches of the right lower lobe pulmonary artery consistent
with PE. Subsequent evaluation revealed the presence of the Factor V Leiden mutation.
DISCUSSION
Tests with high sensitivity rule out (Snout) and high specifity rule in (Spin) diseases.
These, however, are strongly influenced by the prevalence of the disease in the studied
population. Using published clinical prediction rules, this patient has a high pre-test
probability of a PE (> 70%). The sensitivity of the D-Dimer ranges from 85%–99% and
the specifity from 45%–68%. Based on these, the negative likelihood ratio (LR-) would
range from 0.33–0.15 and the post test probability from 35%–1.5%. Given the potential
risk of mortality from a PE (26% if untreated), applying an invasive gold standard
test (pulmonary angiography) is appropriate. The case illustrates the limitations
of the D-Dimer assay in this particular diagnostic strategy. D-Dimer is the primary
product of the enzymatic degradation of cross-linked fibrin by plasmin and is elevated
in the presence of thrombosis. However elevated D-Dimer levels are not limited to
PE or DVT, and the absence of elevated D-Dimer levels does not always rule out PE.
While the D-Dimer assay has a high sensitivity, its negative predictive value depends
upon the pre-test probability of disease, and must be evaluated within the clinical
context.
FORMULARY CONVERSION PROGRAMS POSE A SIGNIFICANT RISK TO PATIENT SAFETY
S. Singh
1; R. Shrivastava1; V. Das1. 1Unity Health System, Rochester, NY. (Tracking ID #116917)
LEARNING OBJECTIVES
Recognise that potential for adverse outcomes exist when formulary conversion programs
are implemented without adequate post-conversion pharmaco-vigilance; and understand
the differences in the pharmocokinetic properties of statins which affect their potential
for interaction with other drugs.
CASE
A 74-year-old white female was admitted for Pleural effusion. Her medical history
included orthotopic cardiac transplantation for ischemic cardiomyopathy, hypertension,
hypercholesterolemia, peripheral vascular disease, diabetes mellitus and renal insufficiency.
She was on a stable dose of cyclosporine, azathioprine and atorvastatin.Atorvastatin
20 mg was switched to simvastatin 40 mg on admission because of formulary restrictions.
On the 12th day the patient complained of generalised weakness. She gradually developed
increasing weakness of her limbs requiring ICU monitoring. Her renal function deteriorated
further requiring dialysis. A peak CK level of over 10,000 mg/dl was noted by Day-29.
Cyclosporine levels which were normal earlier peaked at 681 ng/ml on Day-29. Statin
induced rhabdomyolysis was diagnosed. She died a few days later.
DISCUSSION
CYP3A4 is responsible for more than half of clinically significant drug interactions
seen in clinical practice. Lovastatin, simvastatin and atorvastatin are metabolized
by CYP3A4 while pravastatin and fluvastatin are not. Cyclosporine is a potent inhibitor
of CYP3A4 increasing the risk of myopathy induced by statins using CYP3A4 pathways.
In this case automatic substitution from a stable dose of atorvastatin to a higher
dose of simvastatin contributed to the outcome as statin related myopathy is known
to be dose dependant. Additional risk factors for myopathy included age, female sex,
and renal disease. Health Care organizations' use of formulary conversion programs,
such as an automatic drug substitution policy to maximize resources raise several
concerns regarding patient safety. This case demonstrates the need to screen for drug-drug
interactions, contraindications, and appropriate dosage conversion to minimize risks
of Adverse Drug Reactions while implementing a formulary conversion program. On-going
provider education, provisions allowing physicians to use their judgment in using
a particular drug overriding the substitution policy, and better institutional pharmaco-vigilance
should be integral parts of any safe drug substitution policy.
FUSOBACTERIUM PERICARDIAL EFFUSION CAUSING TAMPONADE—A RARE ENTITY
S.A. Dharashivkar
1; R. Goodman1; A. Waissbluth1; S. Nix1; S. Goldberg1. 1The Jewish Hospital, Cincinnati,
OH. (Tracking ID #115812)
LEARNING OBJECTIVES
1. Recognize anaerobic pericarditis as an uncommon but potentially fatal condition.
2. Know the management of anaerobic pericardial effusion.
CASE
A 50 year old lady on chronic steroids (5 mg/day) for lupus presented with complaints
of severe chest pain which started four hours prior to presentation. The pain was
worse on deep inspiration, lying down and on activity and improved on sitting. Initial
physical exam revealed a blood pressure of 115/87 mmHg, pulse of 85/min, oxygen saturation
99% and a normal cardiovascular exam. During the ER stay, her pain worsened, blood
pressure dropped to 64/48 mmHg and oxygen saturation dropped to 85% on room air. An
emergent CT scan for suspected pulmonary embolism showed the presence of a large pericardial
effusion. Echocardiogram confirmed findings of pericardial tamponade, and pericardiocentesis
yielded 600 cc of opaque, thick, yellowish brown fluid. Pericardial fluid results
showed 158,000 WBCs (neutrophils 97%), 10,000 RBCs, Glucose <2 mg/dL, LDH 1,850. Gram
stain showed gram-negative rods. The patient was empirically started on piperacillin-tazobactam
and vancomycin. Anaerobic cultures showed a growth of Fusobacterium susceptible to
Ertapenem. The patient was discharged home on intravenous antibiotics and was stable
after two weeks.
DISCUSSION
Anaerobic pericarditis is uncommon and associated with significant morbidity and mortality.
The source of origin is usually either a contiguous source of infection, endocardial
spread, hematogenous seeding or direct inoculation. The high mortality associated
with anaerobic organisms makes it imperative to identify the organism quickly to initiate
the right antibiotics. A search for the source of infection should be done. In our
patient no obvious source of infection was identified. An oral panoramic scan which
was planned after discharge could not be done as the patient did not follow-up. Her
immunocompromised status due to chronic steroids predisposed her to this unusual condition
most likely caused by oral anaerobes. Appropriate treatment with pericardiocentesis
and antimicrobials had a good outcome in this potentially fatal condition. To the
best of our knowledge, of the six reported cases of Fusobacterium pericarditis including
this one, this is the second case where the patient survived.
GENES! ANSWER TO AN ELUSIVE CASE OF RECURRENT ABDOMINAL PAIN
S. Daya
1; F.V. Caplan1; D.T. Francois1. 1York Hospital, York, PA. (Tracking ID #116792)
LEARNING OBJECTIVES
1) Recognize familial mediterranean fever as a etiology of recurrent abdominal pain.
2) Recognize the right testing to make the diagnosis following clinical suspicion.
3) Recognize the significant reduction in morbidity and prevention of a life-threatening
complication with institution of therapy.
CASE
A 36 year old male physician of Ashkenazi Jewish descent presented to the office with
more than 20yr history of recurrent self-limited episodes of abdominal pain. Pain
was characterized by insidious onset of incapacitating central abdominal pain along
with right sided pleuritic chest pain. There was associated “sensation” of fever with
no documented raise in temperature. The episodes apparently had started as a teenager.
Ibuprofen was the only medication thought to relieve pain. He was diagnosed to have
irritable bowel syndrome after extensive workup of his symptoms upto this stage. The
patients brother was being investigated for similar symptoms at a university center.
During his last episode, abdominal exam by his physician spouse was remarkable for
diffuse rigidity and decreased bowel sounds. Laboratory investigation was significant
for a microcytic anemia with low iron indices and a raised CRP and sedimentation rate.
Endoscopic evaluation for anemia was unremarkable. After careful review of the patients
history and progress, a syndrome of hereditary periodic fever was entertained. He
was referred to National Institutes of Health for genetic testing of familial Mediterranean
fever. A homozygous familial mediterranean fever (FMF) mutation V726A was revealed
confirming the diagnosis. Following the institution of therapy with colchicine the
frequency of episodes of abdominal pain diminished significantly.
DISCUSSION
Familial mediterranean fever is a genetic disorder inherited as an autosomal recessive
trait, prevalent in individuals of jewish descent. The gene named MEFV(MEditerranean
FeVer) is located on short arm of chromosome 16 and encodes a protein called pyrin
or marenostrin. The expression of the protien is considered to be most likely restricted
to mature neutrophils and appears to act as an intranuclear regulator of transcription
of peptides involved in inflammation. The clinical presentation is characterized by
sporadic, acute self-limited attacks of fever often accompanied by inflammation of
serosal surfaces like peritoneum or pleura and erythematous skin lesions. Diagnosis
is made in individuals of appropriate ethnic background, history and confirmation
by genetic analysis. Treatment is effected by colchicine, which significantly reduces
the number of acute attacks and prevents AA amyloidosis which dictates life expectancy
and prognosis. Genetic testing is a relatively new modality used for diagnosis of
FMF. Early diagnosis and institution of colchicine can prevent significant morbidity
and a life threatening complication.
GUAIFENESIN AND EPHEDRINE NEPHROLITHIASIS
R.S. De Jesus
1. 1Mayo Clinic, Rochester, MN. (Tracking ID #116979)
LEARNING OBJECTIVES
1. Identify features of guaifenesin and ephedrine stones. 2. Recognize data from history
that give rise to this entity. 3. Alert clinicians to yet another sequelae of ephedrine/guaifenesin
abuse.
CASE
A 37 year old Caucasian female presented with approximately 5–6 hour duration of right
flank pain that radiated to her right groin and nausea. There was no vomiting, fever,
chills, dysuria or urinary urgency. Her medical history was significant for recurrent
uric acid stones, renal tubular acidosis and prior lithotripsy. She had smoked one
pack per day for 20 years but had only minimal alcohol use. She had a remote history
of substance abuse. Vital signs on admission were T: 36.7, PR: 85/min., RR 18/min,
BP: 130/68. There was right CVA tenderness and right inguinal discomfort on palpation.
Laboratory tests showed no leukocytosis, normal creatinine and uric acid levels. Urinalysis
showed 51–100 rbc/hpf and 4–10 wbc/hpf. A CT of the abdomen and pelvis showed a focal
area of high attenuation in the distal right ureter proximal to UV junction, which
measured 5 mm; there was perinephric stranding, right hydroureteronephrosis and the
presence of several bilateral renal stones. She was dismissed to outpatient follow-up
with scheduled cystoscopy and right ureteroscopy. She returned to the clinic three
days later bringing with her several stones, which she had passed out at home. Analysis
revealed guaifenesin metabolites as well as traces of ephedrine. She subsequently
admitted to taking “cold medications”.
DISCUSSION
Guaifenesin and ephedrine induced renal calculi have only been recently described
in the literature as a novel form of drug-induced renal stones in patients who consume
large amounts of over-the-counter preparations containing these two substances for
use mainly as stimulants. The stones are light tan in color and have radiographic
properties similar to those of uric acid, which may lead to diagnostic confusion.
They are radiolucent on standard X-ray imaging but can be demonstrated on unenhanced
CT. Stone analysis using high performance liquid chromatography would show 70% beta-2-methoxyphenoxy-lactic
acid (a guaifenesin metabolite) and only 5% ephedrine. The exact mechanism of stone
generation has yet to be elucidated but hypocitraturia is thought to play a role.
The initial management is similar to that of individuals with other types of calculi.
An opportunity is given to pass ureteral sones spontaneously before resorting to minimally
invasive techniques such as ureteroscopic extraction or shock-wave lithotripsy. Substance
abuse counseling is strongly recommended after stone passage or removal to prevent
future recurrence.
HEMODIALYSIS FOR VALPROIC ACID OVERDOSE
T. Siddiqi, MD
1. 1The University of Connecticut, Farmington, CT. (Tracking ID #116033)
LEARNING OBJECTIVES
To illustrate the effectiveness of hemodialysis (HD) in clinically severe overdose
of valproic acid (VPA).
CASE
A 43 year old woman, with a history of bipolar disorder, opioid anelgesic drug addiction
and alcohol abuse, was admitted with obtundation following a suicide attempt with
VPA ingestion (up to 60g). On admission she was comatose (GCS 6), hypotensive, and
had fixed dilated pupils. Her initial VPA level was >1500 mg/l. She was hypocalcemic
with prolonged QTc in her EKG. Her CBC, chem 7, NH3 level and LFTs were within normal
limits. Imaging of the head and brain was unremarkable. She was intubated for airway
protection and respiratory depression. 2 doses of charcoal were administered for gastrointestinal
decontamination. HD was started within a few hours of presentation. 3 hours into dialysis,
her VPA level was found to be 1107 mg/l and dropped further to 591, 261, and 182 mg/l
with continuing HD (T1/2=2.6 hrs). The following day, it was 148 mg/l, and finally
70 mg/l. Further HD was not required. On awakening and extubation a few days later,
the patient underwent a psychiatric evaluation and was subsequently transferred to
a psychiatric facility.
DISCUSSION
VPA is an anti-epileptic agent that is widely used for prophylaxis of bipolar disorders
and migraines. Its toxicity is being reported to poison control centers with increasing
frequency in recent years. Severe symptoms include coma, respiratory depression, hemodynamic
instability, multi-organ failure, and death. VPA's low molecular weight (144 daltons)
and low volume of distribution suggest a potential benefit of HD. Although there is
a high degree of protein binding, in severe toxicity proteins are saturated and most
of the drug is found in its free form in the body. Thus, efficacy of HD increases
in this situation. However, there is limited data guiding the use of HD for extracorporeal
removal of VPA in severe overdose cases. Among the handful of reported cases of HD
use (with or without hemoperfusion) for VPA toxicity, there has been a >60% success
rate. Elimination half-life was markedly reduced in these cases. A similar effect
was noted in our patient. Controlled trials are required to assess whether removal
of VPA by HD significantly improves outcome among overdose patients.
JUST STUNG BY A BEE ... WHAT WENT WRONG
T. Thenappan
1; S. Parikh1; P. Kapoor1; K. Shankar1; H. Friedman1. 1St. Francis Hospital, Evanston,
IL. (Tracking ID #115559)
LEARNING OBJECTIVES
1.Diagnose Henoch Schonlein Purpura (HSP) as a late complication of bee sting. 2.Recognize
HSP in an adult patient presenting with purpuric rash, abdominal pain and arthralgia.
CASE
A 41-year-old Caucasian male, with no significant past medical history, presented
with a non pruritic rash of both the legs for three weeks. The rash started one week
after a bee sting. Subsequently, he developed diffuse, crampy abdominal pain accompanied
with swelling of the knee and ankle joints. The physical examination was notable for
epigastric tenderness and a palpable purpuric rash in both the lower extremities.
His stool was positive for occult blood. Laboratory investigations revealed leukocytosis
(20,000 cells/cumm), elevated ESR, microscopic hematuria and proteinuria. The CT scan
of the abdomen showed marked thickening of the third and fourth parts of the duodenum
and the proximal ileum. Subsequent upper endoscopy visualized multiple linear deep
serpiginous ulcerations in the duodenum. The skin biopsy of the purpuric rash demonstrated
leukocytoclastic vasculitis associated with fibrinod deposits. His serological work
up for hepatitis B and C were negative as were the HIV, EBV, CMV and Mycoplasma titers.
ANA, ANCA and cryoglobulins were within normal limits. The diagnosis of HSP was established
based on the skin lesions and joint, renal and gastrointestinal involvement. The patient
responded remarkably to intravenous corticosteroids.
DISCUSSION
HSP, also referred to as anaphylactoid purpura, is a diffuse systemic, small vessel
hypersensitivity vasculitis occurring predominantly in children. It is characterized
by non-thrombocytopenic purpuric rash of the lower extremities, arthralgia, abdominal
pain and renal involvement. The presumptive pathogenic mechanism is IgA dominant immune
complex deposition in venules, capillaries and arterioles. The common inciting antigen
is an upper respiratory infection. However, various drugs, food, immunization and
insect bite have also been suggested. In our patient, in the absence of other triggers,
the most probable precipitating factor is the bee sting. A small percentage of patients
with HSP progress to renal failure. Therefore, diagnosing HSP as a late complication
of bee sting is important. Close follow up with repeated urinalysis is warranted.
HSP, a disease once thought to be confined to children is increasingly being diagnosed
in adults. This case underscores the need to consider classic syndromes even in patients
who, because of age or other demographic factors, are at relatively low risk.
HEPATIC SARCOIDOSIS
J.A. Kasher
1. 1UCLA San Fernando Valley Program, Sylmar, CA. (Tracking ID #116588)
LEARNING OBJECTIVES
1) Recognize clinical presentation of hepatic sarcoidosis. 2) Recognize hepatic sarcoidosis
as an unusual cause of end-stage liver disease.
CASE
45-year-old female presented with acute upper gastrointestinal bleeding as well as
complaints of progressive jaundice, fatigue, pruritis, and unintentional weight loss
over a one year period. Endoscopic evaluation revealed esophageal variceal bleeding
which was treated with banding. Examination revealed jaundice as well as several stigmata
of chronic liver disease including hepatosplenomegaly, mild ascites, prominent superficial
abdominal veins, hemorrhoids, malar telangiectasias, and palmar erythema. Also present
were numerous skin excoriations and lichenification due to scratching. The remainder
of the physical exam, including assessment of neurologic status, was unremarkable.
Her liver tests showed total bilirubin 2.9, alkaline phosphatase 544, AST 52, and
ALT 62. Other routine chemistries, blood count, and chest x-ray were normal. Liver
biopsy showed cirrhosis, non-caseating granulomas, bile duct obstruction, and small
bile duct loss. Fungal and acid fast bacillus cultures showed no growth. Mitochondrial
antibody titers were negative, and angiotensin converting enzyme (ACE) level was elevated
at 92 (normal 9–67). The diagnosis of hepatic sarcoidosis was made. Subsequently,
the patient's hepatic dysfunction progressed over the next few months and she developed
encephalopathy. The patient consequently underwent liver transplantation and clinically
improved. Six months postoperatively, her course was complicated by CMV infection
of the liver, but no recurrence of granulomatous disease was present on repeat liver
biopsy.
DISCUSSION
Sarcoidosis is a disease of unclear etiology characterized pathologically by the presence
of non-caseating granulomas. Pulmonary involvement is present in 95% of cases, but
many other organ systems can be affected. About 11% of patients have hepatic involvement.
Most cases are subclinical, and symptomatic liver involvement is reported in less
than one percent of patients with sarcoidosis. Patients with symptomatic hepatic sarcoidosis
usually present with fever, malaise, weight loss, jaundice, and pruritis. Chest x-ray
may not necessarily show evidence of pulmonary involvement. Hepatosplenomegaly is
common, and elevation of alkaline phosphatase is commonly seen. A Kveim-Siltzback
skin test may be positive, and mitochondrial antibody test is negative. ACE level
is elevated in about 75% of cases. These findings help differentiate sarcoidosis from
other causes of granulomatous hepatitis including mycobacterial, fungal, and toxoplasma
infections, drug reactions, Crohn's disease, primary biliary cirrhosis and Hodgkin's
disease. Hepatic sarcoidosis may spontaneously improve or may show a relentless progression
as occurred in this patient. Prednisone may be of benefit in the treatment of hepatic
sarcoisosis, and liver transplantation remains an option in cases of end-stage liver
disease.
HEPATIC SINUSOIDAL DILATATION WITH ISOLATED ELEVATED ALKALINE PHOSPHATASE AS PARANEOPLASTIC
SYNDROME IN HODGKIN's DISEASE
S. Habis
1; L. Cation1. 1University of Illinois at Peoria, Peoria, IL. (Tracking ID #116257)
LEARNING OBJECTIVES
To recognize the clinical presentation of Hodgkin's disease despite the absence of
lymphadenopathy. To recognize the hepatomegaly and elevated alkaline phosphatase as
paraneoplastic effect of Hodgkin's disease.
CASE
21 years old male presented with weight loss, severe fatigue, and night sweats of
several months duration. No past medical history. Physical examination was remarkable
for hepatomegaly and no lymphadenopathy. Laboratory evaluation revealed hemoglobin
9.5 g/dL, MCV 71.6 FL, normal WBC and differential, albumin 2.6 g/dL, ANA 1:640 speckled,
ESR 131 mm/hr, and alkaline phosphatase 330 U/L. Chest X-ray was normal. CT scan of
the abdomen revealed hepatomegaly without distinct mass, no lymphadenopathy. Iron
saturation was 6.5%. The following subsequent tests were normal or negative: urinalysis,
PT/INR, PTT, HIV Ab, TSH, CMV PCR, anti-DNA Ab, SSA, SSB, SCL-70, RNP, anti-mitochondrial
Ab, anti-smooth muscle Ab, anti-gliadin Ab, anti-endomysial Ab, anti-phospholipid
Ab screen, bone scan, small bowel follow-through X-ray. Upper and lower GI endoscopy
revealed mild gastritis, small pre-pyloric ulcer with upper and lower GI biopsies
negative for malignancy. The patient was started on Omeprazole and iron therapy. Over
the next 3 months he felt progressively worse. Repeat labs were unchanged. A bone
marrow biopsy revealed no malignancy. A liver biopsy revealed prominent sinusoidal
dilatation and no malignancy. A repeat chest X-ray was interpreted as having mediastinal
widening and a chest CT revealed a mediastinal mass. Biopsy of a lymph node revealed
Hodgkin's disease. The patient was started on chemotherapy and his hepatomegaly and
alkaline phosphatase elevation resolved at the completion of therapy.
DISCUSSION
Lymphoma was high on the differential diagnosis list for this patient at presentation
as patients with lymphoma commonly present with fatigue, night sweats, and weight
loss. The initial normal chest X-ray, hepatomegaly, and elevated alkaline phosphatase
prompted an exhaustive, expensive, and time-consuming search for a hepatic or GI etiology
to his complaints. An earlier chest CT scan may have revealed the diagnosis closer
to his presentation. The paraneoplastic effects of Hodgkin's disease are less common
but can involve many organs including the liver. The finding of an elevated alkaline
phosphatase and liver sinusoidal dilatation have been described in the setting of
Hodgkin's disease. An unexplained elevated alkaline phosphatase should prompt the
primary care physician to consider Hodgkin's disease in the differential. Our case
illustrates an uncommon presentation of a common malignancy and the pearls and pitfalls
in the evaluation of a suspected lymphoma case.
HIV & GLOMERULUS—IS RENAL BIOPSY NEEDED?
S. Parikh
1; P. Kapoor1; T. Thenappan1; H. Friedman1. 1St. Francis Hospital, Evanston, IL. (Tracking
ID #102039)
LEARNING OBJECTIVES
1. Identify HIV associated immune complex diseases (HIVICDs) with its relatively good
prognosis as distinct from the classic HIV associated nephropathy (HIVAN). 2. Type
I membranoproliferative glomerulonephritis (MPGN) can occur in patients infected with
HIV without hepatitis C virus (HCV) co-infection.
CASE
A 28-year-old African American male presented with progressively increasing shortness
of breath and generalized swelling of one week duration. His blood pressure was 174/100
mmHg. The physical examination revealed anasarca and bilateral crackles on auscultation
of the lungs. The serum creatinine was 1.6 mg/dl and serum albumin was 2.1 mg/dl.
The urine dipstick revealed 4+ protein and 11 RBCs/ hpf but no casts. A 24 hour urine
collection demonstrated 6.25 gm of protein and a creatinine clearance of 55 ml/min.
The renal sonogram showed normal sized kidneys. C3 and C4 levels were normal. Serum
cryoglobulins, HCV and Hepatitis B antibodies were undetectable. HIV status, confirmed
by western blot was positive. His CD4 count was 1154/µl and HIV RNA viral load was
26,000 copies/ml. The kidney biopsy revealed subendothelial and mesangial deposits
on electron microscopy that were positive for IgG and C3 on immunofluoresence staining,
consistent with the diagnosis of Type I MPGN.
DISCUSSION
A wide spectrum of glomerulopathies is associated with HIV infection. HIVAN accounts
for about 90% of such lesions. The classic clinical picture of HIVAN is azotemia,
proteinuria and enlarged echogenic kidneys on sonogram. Hypertension and edema are
conspicuously absent. Histologic features are consistent with focal segmental glomerulosclerosis.
It seems to be related to a direct effect of HIV on the renal epithelium. On the other
hand, HIVICDs account for only about 10% of renal lesions in HIV infected patients.
These include MPGN and IgA nephropathy, which occur secondary to the deposition of
circulating immune complexes against an HIV related antigen in the renal tissue. Type
I MPGN usually occurs in the subset of HIV patients co-infected with HCV. However,
it can occur independent of HCV co-infection, as illustrated by our case. The relatively
less aggressive course of renal disease and good prognosis in HIVICDs contrasts the
rapid course and poor prognosis associated with HIVAN. These features emphasize the
importance of differentiating the two subsets accurately with a renal biopsy. Our
patient was started on an ACE inhibitor, his symptoms gradually improved and proteinuria
decreased to 300 mg/day four weeks later.
HIV INFECTION REVEALED BY RAMSAY HUNT SYNDROME
C. Rathnakumar
1; K. Subramanian1; T. Vallur1; J. Patel1; R. Mills1; A. Grigoriu1. 1Jersey City Medical
Center, Jersey City, NJ. (Tracking ID #117239)
LEARNING OBJECTIVES
Ramsay Hunt Syndrome, caused by Herpes Zoster of the geniculate ganglion, consists
of Ipsilateral facial palsy associated with zoster oticus (herpetic eruptions of the
pinna and some times of the palate & of the occipital region) frequently with deafness.
Herpes Zostercan present as Ramsay Hunt Syndrome in the background of immunodeficiency.
CASE
A 48-year old male with no significant past medical problems, presented with dizziness,
unsteady gait, tinnitus and decreased hearing in the left ear. Symptoms started two
weeks earlier, with tingling sensation and sharp pain in his left ear, followed by
serosanguinous discharge, for which he was treated with an oral antibiotic for one
week, with no benefit. Thereafter, he started to have vertigo, tinnitus & deceased
hearing in his left ear and then asymmetry of his face. On physical exam, the patient
was found to have left peripheral facial palsy, dry dark crusts & wet excoriations
with surrounding erythema of the left external auditory canal, shinny intact tympanic
membrane and left sensorineural deafness. Based on the characteristic clinical presentation,
the diagnosis of Ramsay Hunt Syndrome was made. Patient was treated with a course
of acyclovir and prednisone, in addition to tarsorrhaphy. As a part of work up, HIV
test was done (both ELISA and Western Blot) and the patient was diagnosed to have
HIV positive. The absolute CD4 count (1,349) and CD4/CD8 ratio (1.34) were within
the normal range. Patient was discharged and physical therapy was implemented for
gait training.
DISCUSSION
Ramsay Hunt Syndrome can be the sole manifestation of HIV infection in an otherwise
healthy young adult, as presented in our patient. Physicians should recognize that
it is essential to test for HIV in any person presenting with Herpes Zoster infection,
including Ramsay Hunt Syndrome.
HUMAN IMMUNODEFICIENCY VIRUS ASSOICATED NEPHROPATHY
S. Moparty
1; J. Aliota1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane
University, New Orleans, LA. (Tracking ID #117539)
LEARNING OBJECTIVES
1. HIV infection is associated with multiple types of renal disease 2. The most common
type of HIV associated nephropathy is focal glomerulosclerosis (FGS)
CASE
A 38 year-old man presented with three-months of progressively worsening headaches.
On admission, the cachectic patient was noted to be hypertensive and azotemic with
a blood pressure of 185/98 mmHg and a serum creatinine of 33 mg/dl. The patient was
emergently dialyzed. A renal biopsy was performed revealing collapsing glomerulosclerosis
consistent with human immunodeficiency virus (HIV) associated nephropathy. Upon further
questioning the patient revealed a remote history of intravenous drug use, and admitted
to rare but unprotected sexual relations. The patient tested positive for both hepatitis
C and HIV, with a HIV viral load of 62,000, and a CD4 count of 290.
DISCUSSION
HIV infection is associated with many renal diseases, the most common of which is
focal segmental glomerulosclerosis (FSGS) affecting two to ten percent of all patients
with HIV, is a often accompanied by severe tubulointerstitial damage. This type of
injury can be seen in asymptomatic or primary HIV infection as well as in advanced
disease. The mechanism of HIV-mediated renal cell injury is unknown though it is thought
that the interaction between mesangial cells and HIV-infected CD4 cells may lead to
mesangial hypertrophy and sclerosis. It is postulated that preexisting hypertension
or diabetes accelerates HIV nephropathy, perhaps by further stimulating mesangial
cell hypertropthy. Treatment of this disease centers on HAART therapy and ACE inhibition
(in patients with serum creatinine <2.0 mg/dl), although both of these modalities
lack a well-controlled clinical trial. General internists should be aware of the potential
for aggressive renal disease in HIV-infected patients, and should be particularly
vigilant in caring for HIV-infected patients that also have hypertension or diabetes.
HYPERCALCEMIA AND IMMBOLITY: NEED WE KNOW MORE?
P. Chahal
1. 1Mayo Clinic, Rochester, MN. (Tracking ID #115158)
LEARNING OBJECTIVES
1. Recognize the consequences of prolonged immobilization in hospitalized population
2. Recognize immobilization as one of the etiologies of hypercalcemia
CASE
63 year old male with multiple medical problems was admitted with the history of ankylosing
spondylitis, disseminated coccidiomycosis involving lumbar spine leading to multiple
surgeries of his lumbar spine and prolonged hospitalization for almost three months
was found to have serum calcium of 11.9 mg/dl. His physical examination was unrevealing.
His past medical history was significant for colonoscopic removal of malignant polyp
fourteen months ago. His medications were also noncontributory. Consequently, his
workup of hypercalcemia revealed normal alkaline phosphatase, phosphorous, creatinine,
25 hydroxy vitamin D, serum protein electrophoresis, TSH, Angiotensin converting enzyme
level, parathyroid hormone related peptide, mildly suppressed PTH at 0.4 pmol/L (range
1.0–5.2 pmol/L), normal bone scan. He also underwent extensive workup to exclude malignancy
as etiology with CT scan of chest, abdomen and pelvis, MRI of abdomen revealed two
lesions in liver, which were biopsied revealing benign reactive tissue. He also underwent
repeat colonoscopy, which was also normal. He was started on scheduled physical therapy
sessions and short course of Calcitonin and single dose of Pamidronate with subsequent
normalization of his serum calcium level at one and half month follow-up.
DISCUSSION
Hypercalcemia is frequently encountered metabolic abnormality. The most common etiology
in the hospitalized population is the malignancy. Hence, this fact leads to battery
of laboratory and radiologic tests in pursuit of finding occult or overt malignancy
in the hospitalized population. Evident etiologies like prolonged immobilization leading
to high bone turnover escape physician's attention. It is important to consider prolonged
immobility as one of the etiologies of hypercalcemia. This could avoid unnecessary,
expensive and potentially harmful investigations.
Table 1
Laboratory features of common causes of Hypercalcemia
Laboratory
Malignancy
Hyperparathyroidism
Immobilization
PTH
low
high
low to low-normal
PTHrp
High
Normal
Normal
Vitamin D
Low
Normal
Low or normal
Phosphorous
Loq
Low
normal
HYPERCALCEMIA AND NORMAL CALCIUM ON ADMISSION?
N. Gupta
1; S. Wali2. 1Olive View- UCLA Medical Center/UCLA-San Fernando Valley Program, Sylmar,
CA; 2Olive View-UCLA Medical Center/UCLA-San Fernando Valley Program, Sylmar, CA.
(Tracking ID #117408)
LEARNING OBJECTIVES
1) Recognizing hyperparathyroidism as an unusual cause of acute severe hypercalcemia
in an inpatient setting. 2) Review the main causes of hypercalcemia and their typical
clinical presentations/treatment.
CASE
A 70 y.o. Hispanic female was admitted to the surgical service for elective adrenalectomy
for a 6 cm mass found on CT, suspicious for tumor. Past medical history includes hypertension,
Hepatitis C, and liver cirrhosis. The surgery was complicated by severe blood loss.
Replacement blood products, IV fluids, and FFP were given with secondary development
of iatrogenic pulmonary edema, requiring ICU transfer for intubation and diuresis.
ICU course was further complicated by Pseudomonas pneumonia, and Stenotrophomonas
and Enterococcus sepsis. After 2 weeks in the ICU, the patient was extubated and transferred
to the general medicine team in stable condition, with subsequent development of altered
mental status, nausea, vomiting, and rapidly rising calcium from baseline levels (8.8
on admission, 9.5 on transfer from ICU, peaking to 12.2 over 4 days). Significant
lab values include: Na+= 151; Cr = 1.3; glucose = 154, Mg = 2.2, phosphorus = 2.3;
albumin = 3.0; ionized calcium = 6.2 at peak (normal: 4.4–5.2); PT= 20; INR = 1.8;
intact PTH = 132 (normal: 10–65); 24 hour urine calcium = 418 mg/24 hour (normal:
100–200). A head CT without contrast was negative for any acute CVA/intracranial hemorrhage/mass.
The patient also received a CT chest/abdomen/pelvis, which showed a cirrhotic liver
and a large right subcapsular renal hematoma, but no other evidence of any masses
suggestive of malignancy. EKG was normal. The patient was treated with IV pamidronate
with subsequent resolution of hypercalcemia over 2 days. Furosemide and IV fluid treatment
for hypercalcemia were held secondary to mild hypernatremia. The patient was diagnosed
with hypercalcemia secondary to hyperparathyroidism despite normal calcium levels
on hospital admission.
DISCUSSION
IMPLICATION/DISCUSSION: Hypercalcemia is a relatively common clinical problem. Although
the differential for hypercalcemia is extensive, hyperparathyroidism and malignancy
account for approximately 90% of all cases. Hyperparathyroidism is the most common
cause in an ambulatory setting, accounting for greater than 90% of cases. In hospitalized
patients, cancer is the most common cause, accounting for approximately 65% of cases,
with hyperparathyroidism accounting for approximately 25% of inpatient cases. In primary
hyperparathyroidism, calcium levels are usually only slightly elevated, usually less
than 11 mg/dL and rarely exceed 12.5 mg/dL. Calcium levels greater than the aforementioned
values, are usually attributable to a malignancy. Clinical manifestations include
lethargy, weakness, constipation, pancreatitis, nephrolithiasis, shortened QT interval,
psychiatric disturbances. Treatment includes heavy hydration, lasix for calciuresis,
calcitonin, biphosphonates, and steroids depending on the degree of hypercalcemia.
HYPERTENSION IN PATIENTS WITH UNILATERAL ATROPHIC KIDNEY: AN UNCOMMON ETIOLOGY OF
A VERY COMMON MEDICAL DIAGNOSIS
Y.Y. Li
1; R. Granieri1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115244)
LEARNING OBJECTIVES
1) Recognize the relationship between hypertension and unilateral atrophic kidney
2) Recognize multiple causes of unilateral atrophic kidney 3) Recognize the difference
in the management of hypertension in patients with unilateral atrophic kidney or essential
hypertension.
CASE
A 37 year-old male presented with a 3 week history of mild right flank pain. He denied
dysuria, hematuria, fever, chills, nausea or vomiting. He was not on any medications.
Physical examination was unremarkable except blood pressure was 130/85 mmHg. An abdominal
ultrasound revealed an atrophic right kidney, a compensated left kidney with no hydronephrosis.
The flank pain subsided without further therapy. About 2 years later, the patient
developed hypertension with blood pressure of 150/100 mmHg. MRA showed minimal blood
flow through the right renal artery and a widely patent left renal artery. Additional
studies showed 24 hour urine protein 90 mg, creatinine clearance 93 ml/min, plasma
renin activity 21U/ml (normal 5–13 U/ml; >13 suggests renovascular hypertension),
normal VMA, metanephrine, catecholamines, and aldosterone. Therapy with ramipril was
started, 2.5 mg daily, for 2 weeks, then 5 mg daily, which lowered blood pressure
to below 120/80 mmHg.
DISCUSSION
Unilateral small kidney may result from a variety of causes, including chronic pyelonephritis,
obstructive renal atrophy, renal artery stenosis with ischemia, congenital hypolastic
kidney, tuberculosis, radiation therapy and partial nephrectomy. Hypertension in patients
with unilateral small kidney may be essential or secondary hypertension. The small
kidney in renal artery stenosis is usually associated with difficult to control hypertension.
In animal models of renovascular hypertension (2K1C Goldblatt), the elevated blood
pressure is associated with renin hypersecretion from the underperfused kidney and
overactivation of the renin-angiotensin-aldosterone system. However, elevated plasma
renin activity is found in only 50–85% of patients with renovascular hypertension,
and may be seen in 16% of patients with essential hypertension. Medical therapy should
be the primary management except in uncomplicated fibromuscular dysplasia, which is
usually amenable to angioplasty. One should be cautious in using high dose ACE inhibitors
in patients with high renin state because of tendency of causing profound hypotension,
and in patients with possible bilateral renal artery stenosis because of the risk
of reversible acute renal failure. The present patient developed hypertension several
years after the unilateral small kidney was incidentally found. The hypertension may
be caused by the small kidney because the plasma renin activity was significantly
elevated, which may also account for the hypersensitivity to ACE inhibitor therapy.
HYPERTHERMIC EMERGENCY: A CASE OF POST-CARDIAC ARREST HYPERPYREXIA
J. Silberman
1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116239)
LEARNING OBJECTIVES
1. Review differential for hyperthermic emergencies. 2. Recognize post-cardiac arrest
hyperthermia as a rare, but established clinical entity. 3. Review treatment options
for hyperthermic emergencies.
CASE
A 60 year old Hispanic male with ischemic, dilated cardiomyopathy (EF 20%) complained
of chest pain and was subsequently found unconscious. He presented in a ventricular
fibrillation cardiac arrest. His initial ABG revealed pH 6.24, pCO2 73, pO2 282, HCO3
15.3, saturation of 98% on AC with an FiO2 of 1.0. After an hour long resuscitation,
he converted to sinus tachycardia with 6mm ST segment depressions in the inferolateral
leads. His troponin I peaked at 220. At this time, he had rectal temperature of 108F.
No inhaled anesthetics had been used during resuscitation. His family denied history
of malignant hyperthermia or use of psychiatric medications or illicit drugs. Urine
drug screen was negative. He was packed in ice and administered intravenous dantrolene
with a resultant decrease in his temperature to 103F. He remained hypotensive despite
multiple inotropes and was too unstable to be transported for head CT. He subsequently
developed multiorgan failure. Because of the grave prognosis, his family withdrew
care and the patient expired.
DISCUSSION
The most important causes of severe hyperthermia (temperature greater than 104F) are
heat stroke, neuroleptic malignant syndrome, and post-anesthetic malignant hyperthermia.
Although not well recognized, there is also an established relationship between cardiac
arrest and post-resuscitation hyperthermia. Central nervous system insult is believed
to be the cause of this type of hyperthermia. Two recent studies cited 194 patients
with post-cardiac arrest hyperthermia. In these patients, the hyperthermia was a poor
prognostic indicator, often resulting in brain death. Treatment options for post-resuscitation
hyperthermia are not well established. Our approach was to use methods indicated for
other hyperthermia syndromes. The approach favors discontinuation of culprit medications
and symptomatic relief with ice, fans, cooling blankets, and infusion of cool saline.
Refractory cases may require gastric or peritoneal lavage with cold saline or cardiopulmonary
bypass with external cooling of blood. Pharmacologic therapy with dantrolene may also
be indicated, depending upon the cause. Our case illustrates the importance of prevention
and early treatment of hyperthermic emergencies. It also defines post-cardiac arrest
hyperthermia as an entity that requires further study.
HYPERTHYROIDISM WITHOUT A THYROID GLAND?
C. Burgdorf
1; J. Wiese1. 1Tulane Health Sciences Center, New Orleans, LA. (Tracking ID #117388)
LEARNING OBJECTIVES
1. Recognized that retained thyroid tissue following thyroidectomy can result in recurrent
hyperthyroidism. 2. Distinguish recurrent primary hyperthyroidism from factitious
or exogenous hyperthyroidism.
CASE
A 42 year-old woman presented with insomnia, anxiety, and weight loss. She had been
diagnosed with Graves disease one year prior. She was treated with a subtotal thyroidectomy
because of a large, compressive goiter. Per the operative report, the subtotal thyroidectomy
included the isthmus and the right and left lobes. She was taking no medications and
had been asymptomatic until one week prior to presentation. At presentation, her pulse
was 86 beats/min; the blood pressure was 158/76 mmHg. She had no palpable thyroid
tissue and no tenderness to palpation. She had an evident tremor and was anxious.
Despite the history of the thyroidectomy, repeat thyroid function tests were ordered.
Her TSH was 0.01 and the free T4 was 2.8. The patient was determined to be clinically
and biochemically hyperthyroid.
DISCUSSION
Obtaining an accurate past medical history is instrumental to refining the differential
diagnosis. This patient's case is a reminder, however, that a past history of thyroidectomy
does not exclude the possibility of recurrent hyperthyroidism. Recurrent hyperthyroidism
occurs in two percent of patients with a history of thyroidectomy, usually from small
remnants of the thyroid gland located posterior to the plane of dissection. In these
patients, it is important to evaluate for ectopic thyroid tissue. This tissue can
arise in locations such as the neck, mediastinum and ovary. These sources can be diagnosed
by radioactive iodine total body scan. Physicians must also consider exogenous thyroid
hormone intake and overtreatment of surgically-induced hypothyroidism with levothyroxine
as causes for recurrent hyperthyroidism. Physicians can diagnose surreptitious thyroid
hormone intake by a low thyroglobulin and normal radioactive iodine uptake scan; subclinical
hyperthyroidism can be diagnosed by a low TSH and elevated T3 or free T4 levels. Aside
from diabetes, thyroid disease is the most common of endocrine diseases presenting
to the general internists. Knowledge of these complications is important to the proper
management of the hyperthyroid patient.
HYPOTHYROID HEART FAILURE
J.A. Kasher
1; J. Wheat1; P.J. De Silva1; P.P. Balingit1. 1UCLA San Fernando Valley Program, Sylmar,
CA. (Tracking ID #116171)
LEARNING OBJECTIVES
1) Recognize hypothyroidism as an etiology of heart failure. 2) Identify hypothyroid-induced
cardiomyopathy as an easily treatable and reversible condition.
CASE
A 56-year-old Hispanic male presented with a two-month history of progressive substernal
chest pain, dyspnea, and decreased exercise tolerance. His chest pain occurred both
at rest and with exertion. Past medical history was significant for hypertension,
hypothyroidism, and atrial fibrillation. Patient denied palpitations. There was no
previous or current use of alcohol or other illicit substances. The patient admitted
to non-compliance with his medications for about six months. Vital signs were notable
for heart rate 97 and blood pressure 147/81. Jugular venous pressure was normal. Chest
auscultation revealed bibasilar rales, irregularly irregular heart rate, and an S3
gallop. Routine chemistries, blood count, and cardiac enzymes were normal. EKG showed
atrial fibrillation without evidence of ishcemia. Thyroid stimulating hormone level
was 55.3, and free T4 and T3 were within normal limits. Echocardiography demonstrated
global hypokinesis and ejection fraction (EF) 15%, while cardiac catheterization revealed
clean coronary arteries with EF 35%. A regimen of L-thyroxine 0.125 mg, benazepril
20 mg, and metoprolol 25 mg was started. Initial improvement of his CHF followed,
and on return visit 3 months later, TSH had normalized. He remained free of any signs
or symptoms of heart failure after normalization of his thyroid tests.
DISCUSSION
Thyroid hormone affects many aspects of cardiovascular function including systemic
vascular resistance, heart rate, ejection fraction, cardiac output, isovolumic relaxation
time and blood volume. It also acts directly on the cardiac muscle and is a regulator
of cardiac gene expression. Hyperthyroidism is a well documented cause of heart failure.
However, hypothyroidism is much less common and rarely mentioned in the literature
as a cause for cardiac dysfunction. The exact incidence of hypothyroid-induced heart
failure has not been determined since it does not typically occur in the absence of
other cardiac diseases. Hypothyroid cardiomyopthy is rare, and is often associated
with mxyedema coma. In this case, the patient developed heart failure only when he
became hypothyroid. His blood pressure and heart rate were still relatively controlled
and it is unlikely that they induced a cardiomyopathy. Hypothyroidism and idiopathic
cardiomyopathy were the other possibilities. However, idiopathic cardiomyopathy is
not as reversible as that due to hypothyroidism. This patient responded rapidly to
thyroid replacement and appropriate CHF management. Unlike most causes of cardiac
dysfunction, hypothyroidism represents a reversible etiology and therefore it is important
to exclude hypothyroidism as a potential cause in patients with cardiac failure.
“I CAN'T MOVE”
C. Chen
1; S. Wali1; P.P. Balingit1. 1UCLA San Fernando Valley Program, Sylmar, CA. (Tracking
ID #117488)
LEARNING OBJECTIVES
1. Recognize hypokalemic periodic paralysis (HPP). 2. Recognize the association of
HPP with hyperthyroidism.
CASE
23-year-old male with presented with sudden onset of weakness of his extremities.
The patient was watching TV after eating pizza for dinner, and at 4am he noticed that
he was unable to rise up from a sitting position. By the time he arrived at the ER,
he was unable to walk. He complained of mild anxiety, and denied recent upper respiratory
tract infection symptoms. Prior to the onset of symptoms, the patient denied any unusual
ingestions and did not perform strenuous exercise. The patient denied any drug, alcohol,
or tobacco use. Family history was noncontributory. On physical examination, the patient
was afebrile, normotensive, breathing comfortably, with a heart rate of 68. There
was no goiter present. Neuromuscular examination revealed good muscle mass in the
extremities, normal cranial nerve function and intact sensation. However, proximal
muscle strength was 3/5 though symmetric. Laboratory results included K 3.0 and TSH
1.2. EKG showed normal sinus rhythm with U waves present. 60 mEq potassium replacement
was given with resolution of his deficits. Endocrinology consultation was obtained
for management of newly-diagnosed hyperthyroidism.
DISCUSSION
HPP is a syndrome in which a patient experiences episodes of flaccid weakness or paralysis,
which may be potentially fatal if respiratory muscles are affected. Acute attacks
are often precipitated by exercise, stress, or a large carbohydrate meal and can last
for several days. During acute attacks, sudden potassium shifts toward the intracellular
space may lower serum levels to as low as 1.5 to 2.5. However, potassium levels are
usually normal between attacks. This syndrome may be familial, with an autosomal dominant
inheritance pattern with variable penetrance, but may also be associated with hyperthyroidism.
Asian/Native American males seem to be particularly susceptible to this association,
with an estimated incidence of about 15% to 20% of thyrotoxic patients. The syndrome
can occur with few thyrotoxic symptoms. The mechanism by which hyperthyroidism causes
HPP is not well understood. It is hypothesized that thyroid hormone may increase Na-K-ATPase
activity (which drives potassium into cells), thus precipitating hypokalemia when
increased levels of epinephrine and insulin are present (e.g. from stress, exercise,
large meals). Another hypothesis suggests that susceptible patients have a mutated
calcium channel, its function being diminished during thyrotoxic states. In the familial
syndrome, most cases are due to a defect in the calcium channel of skeletal muscle.
The treatment of thyrotoxic-associated HPP is the same as in euthyroid patients, namely
replacement of potassium. Resolution of symptoms often occurs after 15–20 minutes.
Maintaining a euthyroid state often prevents further attacks. Beta blockers may be
helpful in reducing the severity of attacks.
I THOUGHT IT WAS ACUTE CHOLECYSTITIS! FOOLED BY A LIVER ABSCESS
M. Kanbour
1; R. Granieri1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115750)
LEARNING OBJECTIVES
1.) Identify the clinical features of pyogenic liver abscesses; 2.) Recognize the
treatment of pyogenic liver abscesses 3.) Identify potential underlying causes of
pyogenic liver abscesses.
CASE
CASE INFORMATION: A 73-year-old male presented with 4 days of unremitting, dull right
upper quadrant (RUQ) pain, fever (39.4°C), chills, and anorexia without nausea, vomiting,
or change in bowel habits. He had a history of myocaridal infarction and prostate
cancer. Medications were simvastatin and aspirin. His exam was unremarkable except
for RUQ tenderness, positive Murphy's sign, and rebound. His Hb was 11.9, WBC 22.1,
(85 PMN, 6 bands), albumin 2.5, ALT 133, AST 132, ALK PHOS 177, total billirubin 1.1,
INR 1.2. His right hemidiaphragm was elevated on CXR. CT abdomen/pelvis showed gallstones
without cholecystitis or biliary ductal dilatation, diverticular disease without diverticulitis,
and a 10 cm right lobe liver abscess. He was started on IV ampicillin/sulbactam. An
8 French pigtail catheter was placed under CT guidance and 250 cc of foul smelling,
blood tinged fluid were drained. 5 days later, the fluid culture grew Peptostreptococcus.
Blood cultures remained negative. Clinically, the patient improved dramatically. A
CT on day 5 showed a 2nd communicating abscess, located superiorly. A 10 French drainage
catheter was advanced. The patient did well and was discharged on oral amoxicillin/clavulinic
acid. After 4 weeks of therapy, CT showed persisting abscess despite a well placed
catheter. He was referred for open surgical drainage and cholecystectomy.
DISCUSSION
IMPLICATION/DISCUSSION: Pyogenic liver abscesses present as RUQ pain, tenderness,
fevers, anorexia, and weight loss and can mimic the presentation of acute cholecysitis.
Ultrasonography is cost effective for diagnosis and can be used to guide aspiration.
Empiric antibiotics should be started as soon as an abscess is diagnosed and continued
for 2–4 weeks. The antibiotics should cover E. coli, K. pneumoniae, bacteroides, enterococcus,
and anaerobic streptococci. Drainage is not required in small abscesses that respond
to antibiotics. A percutaneous needle aspiration is required if an abscess <6 cm.
If an abscess >6 cm, however, a percutaneous catheter should be placed for drainage.
If drainage fails, if there are large multilocular abscesses, or if there is an associated
intra-abdominal infection requiring surgery, referral for open surgery is recommended.
Antibiotics alone are effective in only a few patients; most will require aspiration
or drainage. In 85% of cases, an underlying etiology is identified. Most commonly,
gallstones, diverticulitis, and appendicitis are implicated. Less common causes include
biliary infection, stricture, cholangiocarcinoma, gallbladder empyema, Crohn's, perforated
ulcer, trauma, liver biopsy, dental infection, or cryptogenic. In all cases the underlying
cause should be sought and treated.
I'M DIZZY. COULD THIS BE CANCER?
A. Nadimpalli
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117491)
LEARNING OBJECTIVES
1. Recognize the clinical presentation of CML 2. Understand the indications and methods
of treatment of CML
CASE
A 37 year-old man presented with one episode of dizziness minutes after urinating.
He did not lose consciousness, had no vertigo symptoms, and had intact bowel and bladder
function. He had a headache the prior day for which he took aspirin and acetominophen.
He also recounted large melanotic stools over the past five days. He denied hematochezia
or hematemesis. His blood pressure was 120/70; heart rate 120, and he was orthostatic.
His abdomen was soft without hepatosplenomegaly. He had no lymphadenopathy. His rectal
exam was normal; his stool was hemoccult positive. His hematocrit was 19 mg/dl; WBC
170,000; platelets 800,000. The bleeding time was significantly elevated, prompting
platelet aggregation studies. The response to ristocetin was normal; his platelets
did not aggregate in response to ADP. After he was transfused, an EGD revealed a non-bleeding
ulcer in his duodenum. A peripheral smear and bone marrow biopsy were consistent with
CML. He was started on Imatinib mesylate (Gleevec) and evaluated for a bone marrow
transplant.
DISCUSSION
The initial presentation of CML is variable. Fifty percent are asymptomatic until
the end stage of the disease; a small percentage present with a bleeding episode even
in the face of an elevated platelet count. In these patients, the dysfunctional platelets
are due to abnormal von Willibrand factor and Ristocetin co-factor levels or platelet
receptors, as confirmed by a decreased response to pro-coagulant factors, including
ADP, epinephrine and collagen. Both the bleeding times and the platelet aggregation
qualities may be adversely affected in patients with CML Although hydroxyurea and
busulfan have been used for treatment of CML, neither are curative. Interferon alpha
2A induces complete cytogenic remission (i.e. Philadelphia chromosome negative) in
a minority of patients. Bone marrow transplant, while having an initially increased
mortality in the first 18 months, has a better long-term prognosis than chemotherapy.
Imatinab mesylate, an oral pill that inhibits the tyrosine kinase molecule produced
by the aberrant Bcr/Abl translocation, has also been shown to have better hematologic
and cytogenic response than chemotherapy alone. Recognizing CML is important to the
general internist as it is a common malignancy that with the advent of Imatinib mesylate
can be curable.
IBUPROFEN INDUCED LEUKOPENIA
R. Nica
1; E. Warm1. 1University of Cincinnati, Cincinnati, OH. (Tracking ID #115633)
LEARNING OBJECTIVES
1. Recognize the neutropenia as one of the ibuprofen side effects. 2. Manage the ibuprofen
induced neutropenia.
CASE
Ibuprofen is well known to cause gastrointestinal side effects but many other types
of adverse reactions can occur. We report a case of ibuprofen-induced leukopenia that
began shortly after introduction of the medication and resolved completely with discontinuation.
A 78-year-old African American male with no prior significant past medical history
presented for his annual physical exam. He noted only mild pain in his right knee,
presumed to be from osteoarthritis. He was prescribed ibuprofen 600 mg twice a day
for the pain. A CBC drawn at the time of the office visit as a part of a routine battery
of labs was normal. Three weeks later the patient presented to the emergency department
for a minor trauma. On this occasion he was noted to have an abnormal CBC. His hematocrit,
hemoglobin, and platelet levels were normal, but his white blood cell count was approximately
0.3 mm3. In order to avoid any lab error the CBC was redrawn and a blood smear was
analyzed by the pathologist The patient did not have any complaints, the physical
exam was unremarkable, and no other lab abnormalities were noted. He was advised to
stop the ibuprofen. One month later, with no interventions and no complications noted,
the patient's white blood cell count was normal.
DISCUSSION
Ibuprofen is a nonsteroidal anti-inflammatory agent that is widely used for treatment
of pain and fever. Although ibuprofen has a favorable therapeutic risk-benefit ratio,
a number of potentially serious adverse reactions have been associated with its use.
Leukopenia, especially with neutropenia or agranulocytosis, is a rare side effect
occurring at a reported rate of less than 1%. The mechanism of this side effect is
not known. One proposed mechanism is the presence of an antibody that inhibits the
growth of myeloid progenitors in the presence of ibuprofen. As seen in our case, the
leukopenia is completely reversible if the drug is stopped. It is also thought that
the frequency of this side effect is underestimated because of this property. It is,
however, important to recognize the leukopenia before expensive and invasive studies
are undertaken to determine the cause. To our knowledge there have been no reported
cases of ibuprofen specific induced neutropenic complications. As with other drug
related neutropenia, we assume that the risk of complications is increased with the
degree and duration of the neutropenia. Because the mechanism of ibuprofen and NSAID
induced neutropenia is unknown, we decided not to prescribe any NSAIDs for our patient
in the future.
INFLAMMATORY BREAST CANCER OR UNRESOLVED MASTITIS-THE SIGNIFICANCE OF “HIGH INDEX
OF SUSPICION”
K. Ghosh
1; A.C. Degnim1; D.L. Adamczyk1. 1Mayo Clinic, Rochester, MN. (Tracking ID #116695)
LEARNING OBJECTIVES
1) Recognize clinical features of inflammatory breast cancer. 2) Manage a patient
with persistent signs/ symptoms of mastitis.
CASE
A 58 year-old lady presented with a history of redness, warmth and fullness in the
left upper breast for three weeks. She had completed a 10-day course of Clindamycin
few days earlier prescribed by her primary physician for suspected mastitis. Her breast
symptoms improved slightly on the antibiotics but recurred soon after the medication
was discontinued. She also reported that she had noticed a lump in her left axilla
about 7 months earlier that had been evaluated with a fine-needle aspiration biopsy
revealing benign findings. She denied fever, but reported fatigue and night sweats.
Clinical examination revealed an asymmetrically enlarged left breast with an area
of erythema, warmth and fullness extending for about 4 × 8cm in the upper left breast.
Palpation of the breast revealed an ill-defined thickening deep to the area of inflammation
measuring about 4 cm, and a 2 cm lymph node was palpable in the right axilla. Diagnostic
mammogram was negative for abnormality. Ultrasound evaluation revealed skin thickening
with vague areas of shadowing in the deeper tissues, but no definite mass; the axillary
lymph node had benign features. Skin biopsies taken from two areas of inflammation
were negative for malignancy. An ultrasound-guided biopsy of the ill-defined hypoechoic
area demonstrated an infiltrating ductal carcinoma. The patient underwent sentinel
lymph node biopsy that was positive for metastasis, and is currently undergoing neo-adjuvant
chemotherapy.
DISCUSSION
Inflammatory breast cancer is the most aggressive form of primary breast cancer. Clinical
features include breast swelling with erythema, edema, tenderness, induration, and
rapid spread to axillary lymph nodes. Since the early presentation is similar to mastitis,
any non-lactational mastitis should be viewed with suspicion and followed until resolution.
Diagnostic imaging (mammogram, ultrasound, or even breast MRI in select cases) is
indicated if the mastitis does not resolve completely with antibiotics. Since skin
biopsy may be negative in inflammatory breast cancer, image-guided biopsy, or even
open surgical biopsy of deeper tissue must be pursued in suspicious cases, even in
the absence of a well-defined mass lesion.
INTRAPULMONARY PERCUSSIVE VENTILATION (IPV)—A NEWER MODALITY IN THE MANAGEMENT OF
BRONCHIAL ASTHMA AND ATELECTASIS—A PRELIMINARY STUDY
A. Devarajan
1; R. Blejeru1; N. Maddukuri1; P. Venkataswamy1; R. Dharmaji1; D. Flores2. 1Jersey
City Medical Center, Jersey City, NJ; 2Jersey City Medcial Center, Jersey City, NJ.
(Tracking ID #116500)
LEARNING OBJECTIVES
To determine the effectiveness of Intra Pulmonary Percussive Ventilation in cases
of respiratoratory failure requiring mechanical ventilation.
CASE
Case 1. Ms. MM is a 58 year old white female with a history of severe asthma and COPD
admitted in ICU for hypercapnic respiratory failure. After being treated with inhaled
bronchodilators, antibiotics and IV steroids, she improved initially and has later
developed severe respiratory failure with unresponsiveness and low oxygen saturation
(O2 saturation—60–70% on 100% Fi02). Since the patient refused mechanical ventilation,
continuous Albuterol and Ipratropium along with 100% oxygen and IPV treatment were
administered. After 10 minutes of treatment, patient expectorated a large amount of
thick yellow sputum, and soon thereafter her O2 saturation rose to 88–92%. The patient
regained consciousness. The was discharged home in her usual condition several days
after. Case 2. 59 year old AAM with history of hypertension developed intracerebral
hemorrhage and was managed in ICU with intubation and control of hypertension and
Intra cranial pressure. During the 2nd week of ICU stay, the patient developed marked
atelectasis of right lower lobe as was evident in chest X-Ray. The atelectasis did
not respond to usual chest percussions, frequent turnings of the patient, and nebulized
bronchodilator treatment. Intra Pulmonary ventilation treatment was added to the regular
nebulized bronchodilator treatment for 3 days. This resulted in marked improvement
in symptoms and signs, including drastic improvement in X-Ray findings.
DISCUSSION
Essentially, IPV is a form of mechanical ventilation, assists the respiration of patients
with diseases which limit their normal respiration by helping to clear retained secretions
from the lungs and then providing deep breathing to increase oxygen delivery to the
alveoli as well as flushing carbon dioxide from the pulmonary airways. This form of
mechanical ventilation delivers rapid, high flow, mini-bursts (percussions) of Air
or Oxygen into the lungs while simultaneously delivering therapeutic aerosols. IPVloosens
and helps propel deep retained airway secretions upward from the lungs where they
can be more easily expectorated. The two cases of respiratory failure are presented
here to stress the effectiveness of Intra Pulmonary Percussive Ventilation in the
management of bronchoconstriction from Asthma and airway blockage from atelectasis.
IS IT A CASE OF ACUTE CORONARY SYNDROME OR AORTIC DISSECTION?
B. Xie
1; A. Sohnen1. 1University of Pittsburgh Medical Center, Pittsburgh, PA. (Tracking
ID #115517)
LEARNING OBJECTIVES
Learning Objectives: 1) Recognize the signs and symptoms that distinguish acute coronary
syndrome from acute aortic dissection 2) Diagnose acute aortic dissection
CASE
A 59-year-old female with hypertension, diabetes, hypercholesteremia, s/p colon cancer
resection 3 months previously, presented with sudden, severe, dull, substernal chest
pain. The pain radiated to left arm and jaw, and was associated with nausea, diaphoresis
and shortness of breath. Vitals on admission: temperature 36.4 C, heart rate 62 and
regular, respiration rate 20, blood pressure 157/72 right arm and 154/68 left arm.
Remaining physical examination was unremarkable. Labs were significant for H&H 11.5/35.6,
CPK 30, CK-MB 0.3, Troponin I 0.1. EKG showed sinus rhythm with rate 62 and no ST-T
changes in comparison with a previous EKG. Chest X-ray was normal. Patients was initially
diagnosed as having an acute coronary syndrome and started on intravenous nitroglycerin,
morphine and heparin. Four hours after onset (and 3 hours after treatment), the pain
persisted. At that time, patient mentioned that the pain radiated to lower back and
left hip. Repeat EKG, CPK, CK-MB and Troponin I showed no change. Intravenous heparin
was discontinued immediately, and a spiral CT scan of chest was ordered. It showed
a dissection involved both ascending and descending aorta. The patient was immediately
transferred to the operation room for dissection repair. The surgery was a success
and the patient was discharged to home 10 days later.
DISCUSSION
Distinguishing the chest pain of aortic dissection from acute coronary syndrome is
very important. Although both illnesses are catastrophic, the treatments are quite
different. The onset of myocardial ischemic pain is often gradual with an increasing
intensity over time, while in aortic dissection the onset is typically abrupt with
greatest intensity at the beginning. The pain of ischemia commonly radiates to the
neck, upper extremity or shoulder but rarely to lower back or hip. Migratory chest
pain occurs in most patients with aortic dissection. Ischemic pain usually lasts more
than 2 but less than 20 minutes, unless a myocardial infarction is occurring. Persistent
chest pain without EKG changes should raise concern that the pain is not due to an
acute myocardial infarction. A normal chest X-ray and symmetric upper extremity blood
pressures do not exclude the diagnosis of aortic dissection. As in this patient, the
suspicion of the diagnosis of acute aortic dissection is raised when 1) pain is severe,
sudden in onset, with radiation initially to arm and jaw then to back and hip (migratory);
2) pain persists for 3-4 hours without EKG change. Definitive diagnosis of aortic
dissection is made with aortography (standard but uncommonly used) or noninvasive
techniques (commonly used) including CT scanning, MRI and trans-esophageal echocardiography.
IS SINUS NODE DYSFUNCTION AN ADVERSE EFFECT OF INFLIXIMAB?
S. Dodla
1; T. Townley1. 1Creighton University, Omaha, NE. (Tracking ID #115699)
LEARNING OBJECTIVES
1. To identify sick sinus syndrome as a possible adverse effect of Infliximab. 2.
To realize the importance of being aware of the potential side effects of Infliximab
considering its wide spread use in the recent years.
CASE
The patient is a 41-year-old male with three-year history of Rheumatoid arthritis
who was treated with Steroids and Methotrexate without symptomatic improval. He was
later started on Infliximab infusions along with Methotrexate with good relief in
symptoms. Three months after starting the infusion he developed edema of the feet
with pleural effusions and was given the new diagnosis of CHF. One month after that
he was admitted to hospital again because of three syncopal episodes with complains
of “passing out” while asleep and while awake. He was noted to have six-second sinus
pauses with no escape rhythm on electrocardiographic monitoring associated with the
symptoms. Other medications that he was on included Methotrexate, Naprelan, Imitrex,
Remeron, Celebrex, Neurontin, Glipizide, Bumetanide, Lipitor, Roxicet and Insulin
70/30. Infliximab was discontinued and Dual chamber pacemaker was placed. On one year
follow up, he had no more syncopal episodes, symptoms of Congestive Heart Failure
had resolved and pacer interrogation revealed little to no pacer dependence and no
significant bradyarrythmias. Other PMH include DM and Hyperlipidemia.
DISCUSSION
Infliximab is a human murine chimeric anti TNF-a monoclonal antibody, which has been
widely used for the treatment of Rheumatoid arthritis and crohn's disease. Studies
are underway regarding the cardiovascular effects of this drug especially after the
recent clinical trial of 7 deaths out of 101 patients with moderate to severe CHF
and another case of sudden death in a patient without CHF after Infliximab infusion.
Bradycardia is a rare adverse effect of Infliximab. Although Diabetes and Rheumatoid
arthritis have been associated with sick sinus syndrome, patient had these before
and after the episodes of sinus pauses. The presence of no syncopal episodes, no pacer
dependence after discontinuing Infliximab supports that the sinus node dysfunction
could be related to Infliximab. The pathogenesis behind it, the absence of escape
rhythm and confirmation of the correlation between both will need to be further assessed
by future clinical trials
ISOLATED PULMONARY MAC IN HIV PATIENTS: A CASE OF IMMUNE RECONSTITUTION SYNDROME
P. Huang
1; B. Taqui1; M. Keith1. 1Temple University, Philadelphia, PA. (Tracking ID #116211)
LEARNING OBJECTIVES
1. Recognize immune reconstitution inflammatory syndrome as a consequence of initiation
of antiretroviral therapy in HIV patients. 2. Recognize clinical features of reconstitution
syndrome.
CASE
A 40 year old male presented with one week of cough productive of clear, white sputum
associated with dyspnea, fevers and night sweats. Two months prior, he had been diagnosed
with HIV (CD4 7). He had been started on antiretroviral therapy (lamividine, zidovidudine,
and tenofovir) which subsequently improved his CD4 count to 149. On physical exam,
he was afebrile. He had oral thrush, submandibular lymphadenopathy, and crackles in
left upper lung field. Lab data revealed Hgb 6.6, LDH 253. Chest xray revealed diffuse
alveolar and interstitial opacities in bilateral upper lobes, left greater than right.
CT abdomen/pelvis revealed marked prominence of the pancreatic head and scattered
nodular densities at the lung bases, but no retroperitoneal or mesenteric lymphadenopathy.
Bronchoscopy revealed many acid fast bacilli by staining, but no pneumocystis carinii
(PCP) or fungi. Routine cultures were negative. He was started on isoniazid, rifabutin,
ethambutol, pyrazinamide and clarithromycin for mycobacterial infection. After culture
confirmation of Mycobacteria avium complex (MAC), his regimen was simplified to ethambutol
and clarithromycin. At this time, the patient was also diagnosed with immune reconstitution
inflammatory syndrome. He continues to do well.
DISCUSSION
Immune reconstitution inflammatory syndrome is a relatively new entity described in
HIV patients who have been started on highly active antiretroviral therapy (HAART).
It represents a recovery in the immune response leading to an excessive response to
previously latent infections. There are no well defined predisposing factors for reconstitutuion
syndrome. However, it tends to occur in antiretroviral naive patients with low CD4
counts (<50–100). It occurs 4–24 weeks after initiation of HAART. The syndrome typically
is associated with ophthamologic and dermatologic manifestations. It has also been
linked to herpes, MAC, tuberculosis, cytomegalovirus, hepatitis B and C, cryptococcus
and PCP. It causes atypical presentations of opportunistic infections. Our patient
presented with isolated pulmonary MAC infection, seen more often in chronic lung disease
than in HIV. There are only 24 reported cases of isolated pulmonary MAC in HIV patients.
Patients with reconstitution syndrome recover nicely with treatment of the underlying
infection and continued HAART therapy.
IT's NOT OVER UNTIL IT's OVER
K. Jones
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117475)
LEARNING OBJECTIVES
1. To recognize that a normal Physical exam, normal serial EKGs and normal echocardiogram
do not rule out Myocardial infarction 2. To review the timing, specificities, and
sensitivities of cardiac enzymes
CASE
A 38 year-old man with a history of gastritis, panic attacks, and hyperlipidemia presented
with one day of intermittent retrosternal chest pain that occurred after an evening
of cigarette smoking and binge-drinking. The pain was relieved with ranitidine, but
returned within an hour and persisted until presentation. He had no family history
of coronary disease. His physical exam and EKG were normal. He was given pantropazol
that relieved the pain. A troponin I was drawn, but he was discharged prior to its
return. The next morning, the patient's troponin was found to be elevated at 4.74
(normal <1.0). The patient was called and asked to return to the emergency department,
where he stated that he was without pain and felt entirely well. His examination was
normal. A second troponin, 36 hours post-chest pain, was 24.5. His EKG remained normal.
Despite his lack of current symptoms, the patient underwent a left heart catheterization
that showed a complete blockage of his left circumflex coronary artery.
DISCUSSION
By GUSTO criteria, myocardial infarction is defined as two of three criteria: enzyme
elevation, EKG changes, and clinical story. This patient's presentation was suggestive
of myocardial infarction, but the response to a proton-pump antagonist falsely masked
the diagnosis. Pain from myocardial infarction is intermittent, and an intervention
such as a PPI can coincide with the natural resolution of the anginal pain, fooling
the physician into believing the diagnosis is due to gastric reflux. This patient's
past medical history of alcohol abuse and anxiety attacks induced cynicism on the
part of the physician that further clouded judgment. No test on its own has a sufficiently
strong likelihood ratio to confirm or exclude the diagnosis of myocardial infarction.
For this reason, serial EKG's should be used to detect ischemic changes, as this improves
the likelihood ratios of the test (+LR 14; –LR 0.3). Troponin I levels are sensitive
indicators of cardiac damage especially after 36 hours, but may remain normal for
the first twelve hours after cardiac injury (+LR 14; –LR 0.05). This case illustrates
that if there is sufficient pre-test probability to exclude myocardial infarction,
serial EKG's and troponin assays should be obtained. Physicians should be aware of
the coincident cause-effect heuristic that may prevent accurate diagnosis of ischemic
coronary disease, and the effect of moral judgment (i.e., a history of alcohol and
drug use) on their clinical decision-making.
ITCH WITHOUT RASH
T. Tanabe
1. 1University of Pennsylvania, Philadelphia, PA. (Tracking ID #117370)
LEARNING OBJECTIVES
1) Recognize the presenting symptoms of bullous pemphigoid. 2) Assess the differential
diagnosis of pruritis.
CASE
A 74-year-old man with a history of hypertension presented with intensive pruritis
for two weeks. The patient complained of itches on anterior shoulder, axillae, upper
chest, abdomen and lower legs. He could not recall the initial site of an itch but
denied rashes. His medication included amlodipine only, which he had taken for years.
He denied any changes in home environment, recent sun exposure, intake of herbal medicine
and history of herpes simplex. Review of system was negative for weight loss, diarrhea,
and visual changes except for insomnia due to intensive pruritis. Physical exam revealed
multiple scratches on his upper arms, chest, abdomen and lower legs, especially close
to ankles. No bullae, papules, vesicles, erythema or scales were seen. There was no
involvement of mucous membranes. Direct microscopic exam of the skin with potassium
hydroxide preparation was negative for fungus or yeast. Laboratory values revealed
normal white cell counts with normal distribution, normal renal function and normal
electrolytes. The patient was referred to the dermatology clinic. Bullous pemphigoid
was diagnosed by skin biopsy and the patient was treated with systemic corticosteroid
successfully.
DISCUSSION
This is a fairly common presentation of bullous pemphigoid. Initially patients complain
of moderate to severe pruritis without skin lesion and subsequently develop an eruption.
Erythematous, popular lesions may precede bullae formation by weeks to months. Patients
are often misdiagnosed as generalized eczema, scabies, allergic contact dermatitis
or drug reaction. Histopathology and immunology permit a differentiation from scabies,
dermatitis herpetiformis and the distribution of rash allows one to differentiate
from contact dermatitis or atopic dermatitis. It is crucial for internists to obtain
a detailed history including medications, diet, and the presence of similar symptoms
in a patient's family. It is also imperative to examine mucous membranes carefully.
JUGGLING A DIAGNOSIS WITH TWO FEET AND ONE LEFT HAND
J.S. Dubow
1; M. Rotblatt2. 1UCLA Medical Center, Los Angeles, CA; 2UCLA SFVP-Olive View Medical
Center Department of Internal Medicine, Sylmar, CA. (Tracking ID #116172)
LEARNING OBJECTIVES
1. Recognize how to diagnosis and treat pneumocystis carinii pneumonia (PCP) 2. Recognize
the significance of the “two foot and one hand” syndrome.
CASE
A 32-year-old hispanic man was admitted to the hospital complaining of shortness of
breath, fevers and dsypnea on exertion. He also reported recent night sweats, a 15-20
pound weight loss over 6 weeks and a yellowing of his toenails on both feet and fingernails
on one hand. The patient had moved to the United States from Mexico ten years previously
and he admitted to having sexual intercourse with a prostitue 2 years ago. On exam,
the patient was tachypneic and tachycardiac; he had coarse breath sounds and onychomycosis
on all ten of his toe nails and all five of the finger nails on his left hand. The
rest of the exam was only significant for a large tatoo on his torso. The CXR showed
diffuse hazy interstitial infiltrates. His labs were significant for pO2 of 65, a
LDH of 1087 and a normal CBC. Although we did not know the patient's HIV status, based
on our high clinical suspicion for PCP, the patient was started on high-dose trimethoprim-sulfamethoxazole
IV and oral prednisone. An HIV test was later positive with a CD4 count of 33, and
the sputum PCP DFA was positive.
DISCUSSION
Based on this patients' symptoms, chest X-ray, LDH and fungal nail infection, it was
suspected that this patient had PCP and HIV. Fungal infections of all toenails and
fingernails on only one hand (two foot and one hand syndrome) have been shown to be
a nonspecific finding in patients with immune deficiency states and a marker for AIDS.
The reason why it only affects one hand is unknown, but this clinical finding suggests
an underlying immunodeficiency. It usually affects the proximal portion of the nail
and extends distally. This increased our suspicion for PCP and the diagnosis of HIV
in our patient, who had a fairly classic presentation of PCP. The clinical manifestations
of PCP most commonly include fever, cough and progressive dyspnea. The most common
findings on physical exam are fever and tachypnea, with possible crackles or rhonchi.
The most common radiographic abnormalities are diffuse, bilateral interstitial or
alveolar infiltrates. The two most common abnormal laboratory values associated with
PCP are a CD4 count below 200 cells/mm3 and an elevated LDH level. However, specific
diagnosis of PCP requires documentation of the organism in respiratory specimens by
sputum induction or BAL. First line treatment for PCP is trimethoprim-sulfamethoxazole
or pentamidine for 21 days. For moderate to severe cases of PCP (defined as arterial
oxygen tension of 70 mmHg or less and/or alveolar-arterial oxygen gradient of 35 or
greater on room air) it is recommended to use adjunctive corticosteroids. Our patient
defervesced well and was discharged with follow up in the infectious disease clinic.
LANGERHANS CELL HISTIOCYTOSIS
A. Ramakrishnan
1; L. Lu1. 1Baylor College of Medicine, Houston, TX. (Tracking ID #117428)
LEARNING OBJECTIVES
1) Recognize the symptoms and clinical presentation of Langerhans Cell Histiocytosis.
2) Learn the treatment options for Langerhans Cell Histiocytosis.
CASE
A 35 year-old female with no significant past medical history presented with a one
week history of worsening mental status. She was well until one week prior to presentation
when her family noted that the change in her mental status along with a decreased
in her fluid intake. Physical examination revealed normal vital signs and a disoriented
female with diffuse erythematous maculopapular rash on her scalp and multiple ulcerated
lesions on her vagina. The rest of neurological exam was unremarkable. Laboratory
results revealed serum sodium of 157 and a low urine osmolality, which was consistent
with diabetes insipidus. She was treated with hypotonic fluid and vasopressin with
improvement of her sodium and mental status. The patient subsequently underwent biopsies
of her scalp and vaginal lesions. Pathology showed dendritic cells with abundant vacuolated
cytoplasm and vesicular oval nuclei surrounded by eosinophils, neurtophils and lymphocytes,
and these findings were diagnostic of Langerhans Cell Histiocytosis (LCH). With her
new onset of central diabetes insipidus, a MRI of the brain was obtained revealing
a pituitary lesion, consistent with intracranial LCH. She was treated with a combination
of chemotherapy and radiation and had good clinical response.
DISCUSSION
LCH is a group of disorders that represent abnormal proliferation of the Langerhans'
cell. These disorders present more commonly in children, but can occur at any age.
Their clinical presentation can be very variable but these disorders can be divided
into three major clinical entities: unifocal LCH, multifocal LCH, and acute disseminated
LCH. Unifocal LCH is characterized by solitary or multiple osteolytic bone lesions
and prognosis is good. Multifocal LCH can involve numerous organs including the skin
and reticuloendothelial system. In about 20–50% of cases, there is involvement of
the pituitary gland leading to diabetes insipidus. Acute disseminated LCH is a severe
form of multifocal LCH, which usually presents in infancy, is characterized by a diffuse
cutaneous rash, hepatosplenomegaly, lymphadenopathy, pulmonary lesions and osteolytic
bony destruction. Therapy is based on the extent of disease and prognosis for limited
disease is generally very good. Single lesions can be monitored or surgically excised.
Local radiotherapy has also been effective for skin and bony lesions. For more aggressive
disease various chemotherapeutic agents such as vinca alkaloids, etoposide, methotrexate,
prednisone and 2-chlorodeoxyadenosine have been used with varying rates of success.
For patients with refractory disease, allogenic bone marrow transplantation is also
an option.
LEARNING TO BREATHE CORRECTLY: WHAT YOUR MOTHER NEVER TAUGHT YOU
C.J. Amin
1; E.F. Yee1; B.L. Horowitz1; G.H. Murata1. 1New Mexico VA Health Care System/University
of New Mexico, Albuquerque, NM. (Tracking ID #116853)
LEARNING OBJECTIVES
1. Recognize the clinical presentation and differential diagnoses of laryngospasms
2. Discuss the work-up and management of laryngospasms 3. Recognize functional disorder
as a cause of laryngospasms
CASE
A 52 year-old male presented with 2 weeks of subclinical fevers, and 4 days of cough,
hoarseness, stridor and dyspnea. When he talked, coughing ensued immediately, resulting
in stridor and inability to breathe for a few seconds. His past medical history was
remarkable for gastroesophageal reflux disease (GERD) treated with ranitidine. The
physical exam was unremarkable except for stridor. A diagnosis of laryngospasms due
to GERD and upper respiratory infection (URI) was made upon admission, and treatment
begun with humidified air, anti-tussives, prednisone, and lansoprazole. Pertussis
antibody and culture were sent, and azithromycin started empirically. In spite of
significant antitussive and nebulizer treatments given for several days, his laryngospasms
increased to hourly episodes. He was transferred to the MICU where bo-tox paralysis
of one vocal cord, and tracheotomy/intubation were considered. Nebulized morphine,
racemic epinephrine, IV ativan and IV lidocaine all failed to prevent episodes. CT
and x-rays of the head and neck were unremarkable. Laryngoscopy revealed mobile vocal
cords with paroxysmal motion, but no obstruction or lesions. With these findings,
a learned functional voice disorder was diagnosed. Speech therapy taught the patient
to manage spasms by breathing slowly, pursing his lips, and whispering words during
attacks. Symptoms, frequency, severity, and distress of his attacks subsequently all
improved. The patient was discharged on anti-tussive medications, lansoprazole, and
paxil, with speech therapy follow up.
DISCUSSION
Episodes of laryngitis causing laryngospasms are frequently brought on by URIs or
GERD (including subclinical GERD). Bordetella pertussis, anatomical defects, Zencker's
diverticulum, hyperparathyroidism, and cricopharyngeal spasm can also cause symptoms.
Anatomical lesions must be excluded when laryngospasms do not resolve. Work up can
include laryngoscopy, radiological imaging studies, swallowing studies, and EGD with
a pH probe. Treatment depends on the etiology. This patient had a functional laryngeal
dysfunction from voluntary, non-conscious learned behavior (in response to initial
irritation from a URI or GERD). It is important to recognize this disorder as symptoms
are very distressing to the patient and caregivers, and treatment lies in exercises,
behavioral therapy, and speech therapy. Intubation and bo-tox are never used for a
functional disorder. Long acting anxiolytics and SSRI's may help to manage associated
anxiety or panic attacks.
LEFT SIDED HEPATIC HYDROTHORAX IN THE ABSENCE OF ASCITES—3RD KNOWN CASE
M.J. Ashraf
1; S. Ryzewicz1; K.T. Hinchey2. 1Baystate Medical Center/ Tufts University, Springfield,
MA, Springfield, MA; 2Baystate Medical Center, Springfield, MA. (Tracking ID #117323)
LEARNING OBJECTIVES
Background: Hepatic hydrothorax is a known complication in patients with cirrhosis
and ascites. However, hepatic hydrothorax in the absence of ascites (HHAA) is extremely
rare. We describe a case of “left sided” hepatic hydrothorax in the absence of ascites
and review the literature.
CASE
A 52 year old woman with a ten year history of Primary Biliary Cirrhosis was admitted
with symptomatic, recurrent, left sided pleural effusion. Biochemistry analysis revealed
transudative pleural fluid on both occasions. There were no clinical signs of ascites
and ultrasound of abdomen was also negative for ascitic fluid. There was no evidence
of congestive heart failure, nephrotic syndrome or marked hypoalbuminemia and the
TSH was also normal. A CAT scan of lungs post thorancentesis did not reveal any primary
pulmonary pathology as a cause of recurrent effusion. The patient was managed with
thoracentesis for acute symptomatic relief of respiratory distress. Subsequently,
the patient underwent transjugular intrahepatic portosystemic shunt (TIPS) and 4 months
post procedure the patient remained asymptomatic without recurrence of hydrothorax.
DISCUSSION
The pathophysiology of HHAA remains unclear. The proposed mechanism involves transfer
of ascitic fluid into pleural space through small congenital diaphragmatic defects
because of cyclical negative intrathoracic pressure. The diagnosis can be established
by a radioisotope scan which reveals one-way transdiaphragmatic flow of fluid from
the peritoneal to pleural cavity. Liver transplant is the treatment of choice, however,
TIPS is an effective temporary alternative. The literature search revealed 29 reported
cases of HHAA. The majority of them are right sided. Only two other cases of “left
sided” HHAA have been reported so far.
LEFT UPPER QUADRANT ABDOMINAL PAIN IN A PATIENT WITH ULCERATIVE COLITIS: THE UNUSUAL
SUSPECT
D.S. Kazi
1; L. Lu1. 1Baylor College of Medicine, Houston, TX. (Tracking ID #117083)
LEARNING OBJECTIVES
1. Recognize an atypical presentation of pulmonary embolism. 2. Recognize the increased
incidence of thromboembolic complications in patients with inflammatory bowel disease
(IBD).
CASE
A 34 year-old Latino male with a two-year history of ulcerative colitis (UC) presented
with left upper quadrant pain for seven days. He was on prednisone (60 mg) and mesalamine,
but continued to have 12–15 watery bowel movements a day. Seven days prior to admission,
he began to experience sharp, intermittent left upper quadrant pain that was aggravated
by deep inspiration. He denied chest pain, shortness of breath, cough, hemoptysis,
melena and hematochezia. On examination, he was afebrile, tachycardic and breathing
comfortably with an oxygen saturation of 100% on room air. The chest was clear to
auscultation but mild tenderness was noted over the left lower ribs in the mid-axillary
line. There was minimal, diffuse abdominal tenderness without guarding or rebound.
Stool guaiac was negative. Labs were unremarkable and plain films showed a small pleural
effusion at the left base and a few dilated small bowel loops. Our initial diagnosis
was an ulcerative colitis exacerbation; thus his dose of prednisone was increased.
Over the next few hours, the patient reported increasing left upper quadrant pain
with radiation to the left lower chest and aggravated by deep inspiration. Because
of the pleuritic nature of the pain and the increased risk for thromboembolic events
during acute UC flare-ups, a ventilation-perfusion scan was obtained, which showed
an intermediate probability for pulmonary embolism. A helical CT scan revealed multiple,
bilateral pulmonary emboli with one large segmental embolus involving the left base.
The workup for hypercoagulable states (Factor V Leiden mutation, anticardiolipin antibody,
lupus anticoagulant, Prothrombin 20210 mutation, hyperhomocysteinemia, and deficiencies
of proteins C and S and Antithrombin III) was negative. Venous dopplers of bilateral
lower extremities showed no evidence of deep venous thrombosis. Anticoagulation was
initiated and the patient had an uneventful recovery.
DISCUSSION
Patients with IBD have a 1.3–6.4% lifetime risk of thromboembolic events, with the
risk being highest during exacerbations. The pathophysiology is not clearly understood,
but one hypothesis is an increased prevalence of thrombophilic gene mutations in patients
with IBD. Although an uncommon extra-intestinal manifestation of IBD, thromboembolism
is a cause of significant morbidity and mortality—about 25% of IBD patients who have
a thromboembolic episode will die during the acute event. Therefore, early recognition
and prompt treatment are crucial. This case illustrates the importance of maintaining
a high index of suspicion for thromboembolic events in patients with inflammatory
bowel disease.
LEFT VENTRICULAR OUTFLOW OBSTRUCTION CONFUSED FOR
J. Aliota
1; S. Martin-Schild2; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA;
2Tulane University, New Orleans, LA. (Tracking ID #117533)
LEARNING OBJECTIVES
1. In a hypovolemic patient, left ventricular outflow obstruction and autonomic dysfunction
can be clinically similar 2. Appropriate imaging is essential in making the diagnosis
of left ventricular outflow obstruction
CASE
A 65 year-old man presented with three weeks of watery diarrhea, weakness, and pre-syncope.
He had a history of hypertension and old lacunar infarctions and had been hospitalized
in a rehabilitation facility up until the time of admission. He was orthostatic with
a supine blood pressure of 129/60 mmHg and standing blood pressure of 75/49 mmHg.
He had no murmurs or carotid bruits. The physical exam was unremarkable. His hypertension
while at the rehabilitation facility was uncontrolled despite the use of a dihydroperidine,
oral nitrates, and clonidine. All blood pressure medications were held and despite
aggressive hydration the patient's symptoms and orthostasis persisted. A trans-thoracic
echo was ordered, but errantly delayed. Considering the patient's old cerebral injury,
neurology presumed a diagnosis of autonomic dysfunction in the absence of a cardiac
etiology. The patient was started on meclizine without improvement. Two days later,
a transthoracic echo showed a significant left ventricular outflow obstruction.
DISCUSSION
The clinical presentation of hypertrophic cardiomyopathy with left ventricular outflow
obstruction can appear similar to autonomic dysfunction if obvious physical exam findings
are absent and appropriate imaging is not acquired. Hypertrophic cardiomyopathy may
be inherited, idiopathic, or a result of long standing hypertension. While a left
ventricular outflow obstruction may be symptomatic at rest, syncope, dyspnea, or weakness
are common when a patient's activity increases left ventricular activity or dehydration
decreases left ventricular volume. In our patient, the diarrhea contributed to a decline
in left ventricular preload, thereby decreasing the left ventricular stretch that
was keeping the left outflow tract patent. Treatment with beta-blockade decreases
myocardial contractility and heart rate allowing an increase in diastole, thereby
minimizing the outflow obstruction by increasing the left ventricular diastolic volume.
These patients should be counseled to avoid strenuous activity and to maintain adequate
hydration. Medications that predominately reduce preload or afterload, such as nitrates,
diuretics, ace inhibitors or clonidine should be avoided.
LEG PAIN AS A PRESENTING SYMPTOM OF CARCINOMA OF THE LUNG
J.L. Wall
1; G. Bryant2. 1University of Cincinnati, Cincinnati, OH; 2University of Cincinnati
College of Medicine, Cincinnati, OH. (Tracking ID #115860)
LEARNING OBJECTIVES
Recognize and diagnose the skeletal manifestations of bronchogenic cancer.
CASE
Introduction: Hypertrophic pulmonary osteoarthropathy (HOA) is a skeletal paraneoplastic
syndrome that is strongly associated with intrathoracic malignancies, especially bronchogenic
carcinoma. We report a case of HOA that was the presenting finding in large-cell bronchogenic
carcinoma. Case Presentation: A 64 year old white male with a past medical history
of oxygen-dependent COPD presented with intense bilateral lower leg and ankle pain.
The pain had been present for approximately 2 years and had progressively worsened.
He had been previously treated by another physician with narcotics without relief
and wanted a second opinion. Of note, the patient smoked 2-3 packs of cigarettes per
day and had done so for approximately 50 years. On exam, the patient was cachectic
and in severe respiratory distress. There was impressive clubbing of all fingers.
Radiologic studies revealed hypertrophic osteoarthropathy of the tibial bones bilaterally.
A chest x-ray revealed left upper-lobe infiltrate. On biopsy, this was determined
to be a large-cell carcinoma.
DISCUSSION
Hypertrophic pulmonary osteoarthropathy is a paraneoplastic syndrome particularly
associated with large-cell bronchogenic cancer. Manifestations of HOA include periostitis,
clubbing of the fingers, and synovitis. The long bones are frequently involved and
bone pain is the presenting symptom in approximately 30% of patients. HOA can be primary
or secondary to pulmonary infections, cystic fibrosis, lung neoplasm, cardiac infections
or cardiac malformations. When secondary, treatment is directed at the underlying
etiology. Prognosis varies depending on the primary etiology, but pain remission and/or
recurrence reflect activity of the primary neoplasm. Physicians should be aware of
the clinical presentation of HOA as knowledge of this disease process could lead to
earlier diagnosis of an underlying malignancy.
LESSONS LEARNED FROM AN UNFORTUNATE CASE OF STRONGYLOIDES HYPERINFECTION
R. Pechulis
1; B. Taqui1; C. Veloski1. 1Temple University, Philadelphia, PA. (Tracking ID #116207)
LEARNING OBJECTIVES
1. Recognize risk factors for Strongyloides infection and hyperinfection. 2. Recognize
importance of excluding Strongyloides infection prior to initiation of steroid therapy.
CASE
A 43 year old previously healthy Cambodian male presented with acute worsening of
chronic abdominal pain, painful swallowing and 20 lb weight loss/one month. Exam revealed
cachexia, temporal wasting, oral thrush, periumbilical tenderness and heme positive
brown stool. Labs revealed Na 126, K 4.8, WBC 8.2 (1.9 % eosinophils), albumin 2.2,
and microcytic anemia. HIV test was negative. Gastrointestinal bleeding due to ulcer
or malignancy was suspected. A random cortisol level was 1 mcg/dl and a cosyntropin-stimulation
test confirmed adrenal insufficiency. Hydrocortisone 100 mg IV q8 was started. That
evening, the patient became progressively tachycardic and hypotensive. Blood smear
drawn that morning showed gram negative rods. The patient was transferred to the intensive
care unit, where he developed septic shock, ARDS and intra-alveolar hemorrhage. Multiple
sets of blood cultures grew Enterococcus faecalis, Klebsiella pneumoniae and Bacteroides
thetaiotaomicron. Gastric aspirate and stool samples grew Strongyloides stercoralis
larvae and Ivermectin was initiated. The patient developed DIC, acute renal failure
and cerebral infarct. Due to his grave prognosis, the patient's family wished to withdraw
care and the patient expired on hospital day 18.
DISCUSSION
Strongyloides stercoralis is an intestinal nematode endemic to Africa, West Indies,
South East Asia, South America and southeastern United States. Infection should be
suspected in immigrants and travelers from endemic areas, veterans, and institutionalized
individuals. Chronic infection can be present up to twenty years after travel. Hyperinfection
has been reported in patients with HTLV, HIV, diabetes, renal failure, alcoholism,
steroid use, hematologic malignancy and organ transplantation. Disseminated Strongyloides
hyperinfection presents most commonly with gastrointestinal and pulmonary symptoms.
Other manifestations include nephrotic syndrome and gram-negative sepsis. Eosinophilia,
usually prominent in chronic infection, may be absent in hyperinfection. Its absence
is a predictor of poor prognosis. Hyperinfection mortality is as high as 50–86%. Treatment
consists of ivermectin or thiabendazole and supportive therapy. In retrospect, unexplained
gram negative bacteremia in a malnourished patient from an endemic area should prompt
a search for Strongyloides as the cause. In endemic areas, Strongyloides is always
excluded prior to steroid initiation. Our patient's hyperinfection and subsequent
demise was probably due to malnutrition and steroids. There may have also been underlying
immunosuppression as suggested by the adrenal insufficiency.
LEUKEMOID REACTION IN PATIENT WITH BEHCET DISEASE
D.S. Lababidi
1; L. Cation1. 1University of Illinois at Peoria, Peoria, IL. (Tracking ID #116391)
LEARNING OBJECTIVES
1. Review the clinical manifestations of Behcet's Disease (BD). 2. Review the causes
of and clinical criteria for leukemoid reaction (LR). 3. Report a possible case of
LR associated with BD.
CASE
A 63 yo female with HTN, CAD, asthma, chronic anemia, and long history of BD presented
with stomach perforation secondary to BD. The patient had multiple complications from
her BD in the past including dermatological, ophthalmologic, GI (Lower GI bleeding),
cardiovascular (atheroslerotic CV disease), and renal insufficiency. The patient had
surgery for her stomach perforation and then developed sepsis (slight leukocytosis
13,000 18,000 with fever and hypotension). Thereafter, the patient improved, but had
worsening of leukocytosis and anemia with persistent thrombocytosis. Her highest WBC
value was 30.1 K with 62–95% neutrophils, 6–29% bands, 3–15% metamyelocytes, and 1–4%
myelocytes. Her Hb dropped down from 11.7 g/dL to 8.0 g/dL and her platelets rose
from 347 K to 730 K. All blood, urine, wound cultures were negative. The patient was
on azathioprine and prednisone before hospitalization and during this hospitalization
she did not receive azathioprine. A Hematology consult was obtained when the leukocyte
alkaline phosphatase was found to be high 169 units (15–70). Myeloproliferative disorder
was ruled out and hepatitis C antibody was negative. A diagnosis of LR was rendered
and the patient was started on erythropoieten to improve her anemia. The LR in this
patient was thought to be multifactorial: BD, steroid use, and surgery. The patient's
WBC count subsequently returned to normal, and anemia was improved.
DISCUSSION
Neutrophilia is an absolute blood neutrophil count greater than 7,500 × 106/l in adults.
The most common reason for neutrophilia is inflammatory disease due to microbial infection,
ischemic, autoimmune, or traumatic (like surgery) injury, and adrenergic stimulation
(demargination). When there is combination of more than one factor, very high neutrophil
counts can be encountered as well as immature myeloid forms in the blood, causing
what is known as LR. This is a reaction that resembles leukemia but is due to other
conditions such as infection, stress, surgery, etc. As with leukemia, a person with
LR has a disorganized proliferation of immature white blood cells in the blood and
bone marrow. The main differential diagnosis of mature neutrophilia is CML, LR or
other myeloproliferative disorder. Leukocyte alkaline phosphatase should be normal
or high in a LR and low in CML. Absence of leukemic blast cells in the blood or marrow
differentiates LR from AML. Behçet's syndrome is a systemic vasculitis of unknown
etiology with many clinical manifestations including aphthous ulceration, arthritis,
skin lesions, thrombo-phlebitis, and cardiac, neurological, and GI involvement. There
is minimal data about the hematological manifestations of BD. Reported possible hematologic
manifestations include myelodysplastic syndrome, Hairy Cell Leukemia, chronic neutropenia,
and one case report of LR. Our patient had many of the clinical manifestations of
BD including a possible related LR.
LIMB LOSS FROM HEPARIN-INDUCED THROMBOCYTOPENIA IN A CARDIAC TRANSPLANT PATIENT
Y. Zafar
1; S. Wang1; E. Warm1; Y. Nikiforov1; J. Palascak1. 1University of Cincinnati, Cincinnati,
OH. (Tracking ID #115680)
LEARNING OBJECTIVES
1. Diagnose heparin-induced thrombocytopenia (HIT). 2. Recognize HIT as an etiology
for catastrophic thrombosis. 3. Prevent HIT-related thrombosis and limb loss.
CASE
A 58-year-old man with ischemic cardiomyopathy and a prosthetic mitral valve underwent
right heart catheterization for cardiac transplant evaluation and was maintained on
a heparin infusion. His platelet counts declined but spontaneously returned to normal
while on heparin. One month later the patient was readmitted for cardiac transplantation
and was started on a continuous heparin infusion. His platelet counts fell over six
days from an initial value of 213,000 mm3 to 87,000 mm3 on the day of transplant.
Post-operative day (POD) 1, the patient received 6 units of platelets for a mediastinal
bleed with an incremental increase of only 24,000 mm3. Platelet counts reached a nadir
of 50,000 mm3 on POD 7, and the patient received additional platelets. By POD 7 he
had developed frank ischemia of three digits on his right hand and all ten toes, and
heparin exposure was discontinued. Only the third of three serial heparin-induced
platelet aggregation studies was consistent with HIT, but the more sensitive platelet
serotonin release assay was negative. The patient was treated with Argatroban starting
on POD 8 but required amputation of both feet due to extensive arterial thromboses.
DISCUSSION
HIT type I is a self-limited, non-immune mediated, mild thrombocytopenia of approximately
10% due to the direct aggregation effect of heparin on platelets. HIT Type I is not
associated with thrombosis. HIT type II, seen in 1–3% of patients treated with unfractionated
heparin, is a clinical diagnosis based on a 50% reduction in the platelet count within
5–10 days of heparin exposure. Thrombocytopenia may occur within 12 hours of re-exposure
to heparin. The incidence of HIT is increased in patients having undergone cardiopulmonary
bypass. HIT may result in severe thrombocytopenia due to antibodies formed against
the heparin/platelet factor 4 complex on the platelet membrane. Activated platelets
release thrombogenic microparticles that produce a severe hypercoagulable state and
consequent arterial and/or venous thrombosis. Laboratory testing is supportive. Heparin-induced
platelet activation assays are less sensitive than the platelet serotonin release
assay, but both may give false-negative results. Treatment involves immediate discontinuation
of all sources of heparin. Direct thrombin inhibitors are indicated for anticoagulation.
Platelet transfusions are relatively contraindicated as they may promote thrombosis.
This case underscores the need to monitor platelet levels in patients receiving heparin.
LYMPHOMA PRESENTING AS CARDIAC TAMPONADE
M.S. Patil
1. 1Stoger Hospital of Cook County, Chicago, Chicago, IL. (Tracking ID #116260)
LEARNING OBJECTIVES
1) Recognize that acute pericardial effusion may be the presenting symptom of a non-pericardial
neoplasm. 2) Use imaging studies of the chest early in the course of the effusion
to look for underlying malignancy.
CASE
A 26-year-old man was admitted with shortness of breath of two weeks duration. He
had no significant past medical or family history and did not recall a viral prodrome.
He was tachycardic with a blood pressure of 124/94 mmHg. Pulsus paradoxus was present.
EKG showed sinus tachycardia, diffuse ST segment elevation, and PR segment depression
in inferolateral leads. A bedside echocardiogram showed massive pericardial effusion
with tamponade. He underwent emergent pericardiocentesis under fluoroscopy,and 1.5
liters of hemorrhagic exudative fluid was drained. Culture for bacteria and viruses
was negative. Cytology showed no malignant cells. A workup for connective tissue disorders
was unrevealing. The effusion reaccumulated over the next several days despite treatment
with indomethacin. MRI showed pericardial thickening and effusion, pretracheal and
mediastinal lymphadenopathy, and a large heterogenous anterior mediastinal soft tissue
mass invading the pericardium. CT guided needle biopsy of this mass proved it to be
Hodgkin's lymphoma-nodular sclerosis type. The patient is now receiving chemotherapy
for the lymphoma.
DISCUSSION
Upto 23% of new unexplained large pericardial effusions may be malignant. The most
frequent malignancies presenting in this way are lung and breast cancer, and Hodgkin's
lymphoma. CT and MRI of the chest are useful not only for delineation of pericardial
anatomy and loculations, but also for detection of associated neoplasms in the chest.
Neoplasms should be considered and looked for with early imaging in unexplained and
non-resolving effusions.
MANAGEMENT OF ESOPHAGEAL ULCERS IN PATIENTS WITH AIDS
M.J. Hamilton
1. 1Boston City Hospital, Boston, MA. (Tracking ID #116887)
LEARNING OBJECTIVES
Esophageal disease is a common complication in HIV, and it is the second most common
GI disease after diarrhea. It is important to tease out the classic symprtoms of esophageal
disease, most commonly odynophagia. Once the esophagus is implicated in the symptom
complex, the decision must be made whether or not to treat the patient empirically
before further invasive testing. Finally, if upper endoscopy is performed, a treatment
plan must be initiated based on the findings and/or pathology results. This case highlights
diagnosis and treatment strategies for esophageal ulceration in the patient with AIDS.
CASE
A 51 year old African-American male with AIDS (CD 4 count of 4, viral load of 106,000),
not taking HAART medicines, presented to the hospital with odynophagia and weight
loss of one month's duration. He denied abdominal pain, nausea, vomiting, or bleeding.
His physical exam was noteable for a cachetic middle aged man, afebrile, with oral
thrush and a normal abdominal exam. Initial treatment with fluconazole cleared the
oral thrush, however the odynophagia persisted. Upper endoscopy was performed which
revealed Savary-Miller Grade III esophagitis consistent with severe candidial infection
as well as 3 discrete ulcers in the distal esophagus. One ulcer was biopsied in two
locations and the patient was continued on fluconazole and sent home. Patholgy was
negative for infection. The pateint returned to the hospital two weeks later with
continued odynophagia and repeat endoscopy showed the persistence of the ulcers which
were rebiopsied, in six locations. Pathology later revealed CMV, the patient was started
on ganciclovir and had dramatic improvement in his symptoms.
DISCUSSION
Over the course of the last twenty years in studying patients with AIDS, the etiologies
of esophageal ulceration have expanded. The infectious causes primarily HSV and CMV
are well known, however, a noninfectious etiology “Idiopathic Esophageal Ulceration”
or IEU also exists. Dr. C. Mel Wilcox at Emory University in the early 1990's wrote
several articles designed to study the prevalence of each in a cohort of AIDS patients
with esophageal symptoms. Of the 100 patients who underwent endoscopy, 41 had IEU's,
and 50 had CMV. The remainder had HSV or a combination. Wilcox's study highlighted
the importance of obtaining multiple biopsies from an ulcer as the physical appearance
of the ulcers on endoscopy are not sufficient to make a diagnosis. In this case, a
man with severe enough disease to cause a 15 pound weight loss from lack of eating,
was not correctly diagnosed on first endoscopy, and IEU was not considered. Repeat
endoscopy with multiple biopsies allowed for a diagnosis and treatment plan which
in the end reversed his symptoms. A third follow up endoscopy revealed complete resolution
of his ulcerative disease.
MANAGEMENT OF FLUCONAZOLE RESISTANT CANDIDAL ESOPHAGITIS IN AN HIV-INFECTED PATIENT
K. Luce
1; M. Panda1. 1University of Tennessee, Chattanooga, Chattanooga, TN. (Tracking ID
#115219)
LEARNING OBJECTIVES
1. Recognize the increasing prevalence of resistant Candida albicans infections in
HIV-infected patients 2. Discuss the role of intravenous (IV) Caspofungin as an alternative
to IV Amphotericin B 3. Recognize the role of highly active anti-retroviral therapy
(HAART) in the treatment of candidal esophagitis.
CASE
Our patient was a 36 year old female who had been on fluconazole treatment for several
months with continued severe odynophagia and dysphagia of solid foods. Her CD4 count
was 23, with a viral load of >750,000. Patient was non-compliant with HAART. Exam
revealed extensive whitish plaques in the oropharynx. Upper endoscopy revealed severe,
disfiguring esophagitis. Pathology showed only candidal yeast forms. Culture revealed
Candida albicans species only. Treatment was initiated with amphotericin B. Initially
patient tolerated this well with minimal improvement in symptoms. Despite prehydration
efforts patient showed evidence of nephrotoxicity which resolved after stopping amphotericin
B. At this time a trial of caspofungin was initiated to avoid any renal complication
and the recurrent prolonged nature of her infection. In conjunction frequent nystatin
swish and swallow, amphotericin B mouthwash and HAART was intiated. Within a week,
patient reported significantly less odynophagia and dysphagia. We thus elected to
stop intravenous therapy and follow patient's esophagitis on nystatin swish and swallow
and alone. At one month after discharge, patient reported resolution of symptoms and
repeat endoscopy revealed a normal appearing esophagus with negative biopsies. Her
CD4 count also increased to 132.
DISCUSSION
The current treatment of candidal esophagitis in HIV-infected persons is frequently
oral fluconazole followed by amphotericin B as second line treatment. Nonetheless,
recent literature reports increasing frequency of fluconazole and amphotericin B -resistant
Candida species in this patient population. In such cases, there are few options available
to the treating physician. We report response with IV caspofungin in an HIV-infected
patient with fluconazole-resistant Candida albicans esophagitis unable to tolerate
amphotericin B. At least two randomized, double-blind trials have hailed the effectiveness
and tolerability of IV caspofungin. Further, recent data suggests that institution
of HAART with improvement of a patient's immunity has led to improvement and even
resolution of Candida infection. This case supports the use of caspofungin as an effective
agent against candidal esophagitis.Moreover, the striking resolution of our patient's
esophagitis on HAART and nystatin swish and swallow alone supports the premise that
improvement of patient's immune response can be a successful approach to treating
HIV-associated candidal esophagitis.
MAY-THURNER SYNDROME AND DEEP VENOUS THROMBOSIS: AN UNUSUAL RISK FACTOR FOR A COMMON
DISORDER
M. Daly
1; T. Beckman1; D. McNaughton1. 1Mayo Clinic, Rochester, MN. (Tracking ID #103847)
LEARNING OBJECTIVES
1. Recognize May-Thurner syndrome as an unusual but important risk factor for Deep
Venous Thrombosis (DVT) 2. Appreciate the potential variety of risk factors for DVT
in a single patient.
CASE
A 51 year-old female presented with two days of increasing left lower extremity swelling
and pain. Her past medical history was remarkable for recent surgeries, recent prolonged
car and airplane travel, and chronic smoking. She denied a family history of thrombosis.
She denied a personal history of thrombosis, cancer, or estrogen use. Her examination
was remarkable for an edematous, mildly erythematous, and tender left lower extremity.
Laboratory studies revealed a mild leukocytosis with absolute neutrophilia, normal
baseline coagulation studies, and D-dimer elevated above the upper detectable limit.
A chest x-ray was negative. Lower extremity dopplers showed an obstructive thrombus
extending from the left common iliac to the left common femoral vein. Importantly,
the patient's left iliac vein was observed to course between the right iliac artery
and her lumbar spine. Hence, May-Thurner syndrome was diagnosed. The patient then
underwent successful mechanical thrombolysis and iliac stent placement. Finally, she
was initiated on unfractionated heparin as bridging therapy to warfarin anticoagulation.
DISCUSSION
May-Thurner syndrome is a little known risk factor for DVT. This was first recognized
in 1851 by Virchow, who observed compression of the left iliac vein by the overlying
right iliac artery. May and Thurner subsequently described the mechanism by which
this anatomic relationship leads to thrombosis. May-Thurner syndrome should be considered
in all patients with DVTs involving the left common iliac vein. Diagnosing this disorder
is essential, since endovascular management has proven safe and effective in decreasing
morbidity. Notably, this case highlights the challenge of recognizing May-Thurner
syndrome in patients with multiple risk factors for DVT. Moreover, this case underscores
the importance of diagnosing May-Thurner syndrome, since it leads to an unconventional
treatment for DVT.
MENTAL STATUS CHANGES IN A MAN WITH HIV: A CASE OF MYCOSIS PSYCHOSIS
T. Wassenaar
1; J.M. Sosman1. 1University of Wisconsin Medical School, Madison, WI. (Tracking ID
#116768)
LEARNING OBJECTIVES
1). To identify the epidemiology of histoplasmosis. 2). To recognize the common and
uncommon presentation of disseminated histoplasmosis in an immunocompromised patient.
3). To identify new therapeutic approaches and prognostic outcomes.
CASE
A 32 y/o male with a history of HIV/AIDS diagnosed in 2000 (CD4#80/ul, viral load>500,000cps/ml)
only one-week prior was started on antiretroviral therapy and Bactrim prophylaxis,
was admitted with a fever (39.3 C) and a two-week history of mental status changes,
including word finding difficulty and visual/auditory hallucinations. His initial
workup included a head MRI that was unremarkable and a LP that showed 3 nucleated
cells—all lymphocytes, protein 52 mg/dl, glucose 46 mg/dl, cryptococcal Ag negative,
VDRL negative. He was started on broad-spectrum antibiotics pending the LP results.
His admission labs revealed pancytopenia (Wbc 3.7 K, Hgb 10.3 gm/dl, Plt 31K) and
hepatic insufficiency (AST 740, ALT 218, alkaline phosphatase 306, total bilirubin
4.2), all new from labs drawn two weeks prior. Despite negative cultures he continued
to spike fevers, developed worsening hepatic insufficiency (AST 1070, ALT 315, alkaline
phosphatase 739, and total bilirubin 41.0), and was found to be in DIC (haptoglobin
<6, D-Dimer 2–4, fibrinogin 85). A bone marrow biopsy was performed for his persistent
pancytopenia, which revealed granulomas with budding yeast cells and a positive culture
for Histoplasma capsulatum.
DISCUSSION
Histoplasma capsulatum is a fungal spore that grows naturally in soil and is often
picked up by birds and bats and passed on in their feces. Primary infections from
dust inhalation have been reported. Over fifty million people in North America have
reportedly been infected with this fungus, which is endemic in the Ohio and Mississippi
River valleys. The vast majority of these infections go unrecognized. Our patient
had many of the classic presenting symptoms of disseminated histoplasmosis such as
fever, hepatic insufficiency, and anorexia as well as some rare manifestations including
mental status changes, DIC, and severe pancytopenia. The mortality rate of untreated
disseminated histoplasmosis is >80%; but can be decreased to <25% with treatment.
The standard of care for outpatients is treatment with an azole antifungal. However,
IV amphotericin B is usually preferred in patients requiring hospitalization, as is
often the case with disseminated histoplasmosis. Our patient received IV then PO azole
therapy and made a complete recovery.
MIDWEST REGIONAL RESIDENT AWARD WINNER: DIFFUSE ALVEOLAR HEMORRHAGE RESULTING FROM
MARIJUANA
N. Cummins
1; V.T. Martin1. 1University of Cincinnati, Cincinnati, OH. (Tracking ID #115625)
LEARNING OBJECTIVES
1. Recognize diffuse alveolar hemorrhage as a potential complication from inhalational
illicit drug use.
CASE
A 31 year old black male presented with a six hour history of bright red hemoptysis
and dyspnea, which began 30 minutes after smoking marijuana. The patient denied chest
pain, fever, chills, leg swelling, trauma or other ingestions. He had no significant
past medical history and consumed no medications. Physical exam revealed normal vital
signs and diffuse rhonchi in the lung fields bilaterally. A chest x-ray revealed diffuse
bilateral alveolar infiltrates. Initial laboratory exam revealed a Pa02 of 66 mmHg
and hemoglobin of 15.4 g/dl, which subsequently dropped to 12.9 g/dL over the ensuing
twelve hours. Bronchoscopy revealed a diffuse alveolar hemorrhage in all lung lobes.
Autoimmune disease, vasculitis and infection were ruled out as causes for the alveolar
hemorrhage. A urine toxicology screen was positive for tetrahydrocannibinol and cocaine.
Upon further questioning, the patient stated the marijuana may have been laced with
cocaine. The patient was monitored with supportive care only and was discharged two
days later in good condition.
DISCUSSION
Diffuse Alveolar hemorrhage (DAH) can be caused by various autoimmune diseases, vasculitis,
infection and certain medicines. This represents the ninth case report linking DAH
with the inhalation of free base cocaine. Inhalation of cocaine may induce vasoconstriction
of the pulmonary vascular bed or lead to direct alveolar damage resulting in DAH.
To our knowledge there have been no case reports linking marijuana use with DAH and
therefore it remains speculative as to whether this drug could have played a role
in the development of DAH. In conclusion, DAH is a potentially life threatening complication
that should be in the differential diagnosis of dyspnea after inhalation of illicit
drugs.
MITRAL VALVE HEMANGIOMA WITH SUPERIMPOSED INFECTIVE ENDOCARDITIS
U. Ahmed
1; S.G. Khurshid2. 1Saint Francis Hospital, Evanston, IL, Evanston, IL; 2Saint Francis
Hospital, Evanstion IL, Evanston, IL. (Tracking ID #116481)
LEARNING OBJECTIVES
To recognize an unusual presentation of cardiac tumors.
CASE
A twenty-four old previously healthy woman presented with sudden onset of facial asymmetry
and left arm weakness. She had been having fatigue, generalized weakness and fever
for four weeks for which she was being treated with oral antibiotics. Physical examination
revealed a cachectic woman with left lower motor neuron facial paralysis, flaccid
paralysis of left upper extremity and an apical ejection systolic murmur. CT scan
of brain revealed acute ischemic infarction involving right basal ganglia and internal
capsule. Transthoracic echocardiogram demonstrated vegetations on atrial and ventricular
sides of the mitral valve. The patient was treated empirically for suspected infective
endocarditis. Blood cultures revealed no growth. She remained febrile with spiking
fever up to 102. CT scan of abdomen demonstrated multiple infarcts in kidneys and
spleen. Transesophageal echocardiogram showed a pedunculated mass on the anterior
mitral leaflet. The patient was taken to surgery for excision of the mass. Histopathology
showed aggregates of small blood vessels suggestive of mitral valve hemangioma with
a dense neutrophillic infiltrate. Gram stain from the tissue did not show any organism
and bacterial, viral and fungal cultures were negative. The post operative course
was unremarkable.
DISCUSSION
Primary cardiac tumors are rare and their incidence ranges from 0.002% to 0.3% at
autopsy. Benign tumors account for 75% of the primary tumors; among which myxoma accounts
for 50% whereas hemangioma represents only 2.8%. Cardiac tumors can cause obstruction,
valve dysfunction, arrhythmias, pericardial effusions or systemic embolization and
constitutional symptoms. These cardiac lesions can induce endocardial trauma via high-pressure
jets of blood creating a platelet-fibrin nidus that may become infected. Most cardiac
hemangiomas are asymptomatic and are discovered incidentally by echocardiography,
CT, MRI or autopsy. Review of literature shows only two reported cases of mitral valve
hemangioma: first incidentally found on autopsy, and second presented with chest tightness
and palpitations. This patient presented with signs of infective endocarditis and
was incidentally found to have mitral valve hemangioma. The main reason for negative
blood cultures was thought to be prior administration of antibiotics. No similar case
has been reported in literature.
MORE THAN A PAIN IN THE NECK: LEMIERRE's SYNDROME
S. Jain
1; S.R. Ranji1. 1University of California, San Francisco, San Francisco, CA. (Tracking
ID #117395)
LEARNING OBJECTIVES
1. Recognize the clinical features of Lemierre's Syndrome. 2. Institute the appropriate
diagnostic workup in Lemierre's Syndrome. 3. Understand the rationale for aggressive,
lengthy antibiotic therapy.
CASE
LR is a 63 year old female who presented with neck pain. The patient had been homebound
for the past 30 years due to severe agoraphobia and a schizoid personality disorder,
and was only visited by Meals on Wheels and a physician making house calls. She reported
left sided neck pain with movement and subjective fevers for 2–3 days, and denied
chest pain or shortness of breath. On exam, she was febrile, tachycardic, and hypoxic.
She had poor dentition without any obvious abscesses. She was able to move her neck
with pain, but had no frank meningismus. She had shotty anterior cervical lymphadenopathy,
but no cord could be palpated. Neurologic exam was grossly normal. Labs showed a white
blood cell count of 16.3, and lumbar puncture was negative for meningitis. A CT scan
was then performed, which revealed thrombosis of left internal jugular vein with extension
into the left sigmoid sinus and soft tissue swelling in the parapharyngeal space.
This confirmed the diagnosis of Lemierre's syndrome. Subsequently, blood cultures
grew out Staphylococcus aureus sensitive to methicillin. Chest CT did not reveal septic
pulmonary emboli. The patient was treated with intravenous vancomycin and clindamycin
for four weeks and recovered completely.
DISCUSSION
Lemierre's syndrome (also known as postanginal sepsis or necrobacillosis) is septic
thrombophlebitis of the internal jugular vein, most commonly caused by anaerobic organisms
such as Fusobacterium necrophorum. It is rare and affects mostly young adults. The
precipitant is usually a primary dental infection or pharyngitis, followed by invasion
though the parapharyngeal space to the IJV causing thrombophlebitis. Metastatic infections
complications include septic pulmonary emboli (present in up to 85% of cases), septic
arthritis, splenic or hepatic abscesses, and glomerulonephritis. Patients can present
with local neck findings (in 52% of cases), but fever (occurring in 83% of cases)
and pharyngitis (83%) are more common. CT scan with contrast is the recommended diagnostic
test. Treatment with synergistic antibiotics for 3–6 weeks is recommended to prevent
spread of infection. Anticoagulation remains controversial but is not routinely recommended.
If a patient fails antibiotics, surgical ligation or excision of the internal jugular
vein may be necessary. In the antibiotic era, prevalence and mortality has markedly
decreased, but mortality remains high at 6.4%. Our case is unusual in that the inciting
organism was Staphylococcus aureus rather than the more common anaerobes, and in that
the patient had no evidence of septic embolization.
MORE THAN SKIN DEEP: HYPERCALCEMIA ASSOCIATED WITH MALIGNANT MELANOMA
E. Lee
1; H. Jasti1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115959)
LEARNING OBJECTIVES
1. To recognize the role of parathyroid-related protein (PTH-rP) in hypercalcemia
of malignancy 2. To identify an uncommon association between malignant melanoma and
PTH-rP induced hypercalcemia.
CASE
A 32 year old female with a PMH significant for recurring metastatic melanoma to the
left thigh with resection × 2 presented with several days of nausea and vomiting.
She denied fever, chills, cough, diarrhea or other complaints. Physical exam revealed
a thin Caucasian female in mild distress with a 5 cm ulcerated mass on her left thigh.
Initial laboratory results were significant for a calcium of 17.6 mg/dL. Whole body
CT/PET scan revealed a large mass in the posterolateral left thigh and enlarged lymph
nodes in the external iliac and inguinal regions, which on biopsy were negative for
malignancy. Serum PTH-rP levels came back elevated at 17.8 pmol/L. The patient was
treated with intravenous fluids and pamidronate with resolution of her symptoms. At
time of discharge, the calcium level was within normal limits and the PTH-rP level
was nearly undetectable. Several weeks later, the patient returned with similar complaints
and was again treated for hypercalcemia with fluids and pamidronate. Since that time,
she has undergone successful surgical excision of the left thigh mass and has had
no recurrence of hypercalcemia.
DISCUSSION
PTH-rP mediated hypercalcemia, also known as humoral hypercalcemia of malignancy,
is commonly associated with renal, squamous, and bladder cell carcinomas. It is rarely
associated with malignant melanoma. As a structural analog to parathyroid hormone
(PTH), PTH-rP binds to the same receptors. This results in an increase in calcitriol-mediated
bone resorption, an increase in calcium absorption in the distal tubules, and an inhibition
of phosphate transport in the proximal tubules. Diagnosis can be made in a patient
with hypercalcemia and an elevated PTH-rP level. Treatment consists of intravenous
hydration and bisphosphonates. Clinically, PTH-rP levels can have significance, with
higher levels predictive of a shorter median survival time that is independent of
calcium levels. In addition, levels above 12 pmol/L are associated with a decreased
response to bisphosphonate therapy and a greater incidence of recurrence after treatment.
This patient had recurrent hypercalcemia with no evidence of metastatic disease. PTH-rP
induced hypercalcemia should be suspected, and considered a poor prognostic indicator,
in any patient with a solid tumor in the absence of bony metastases.
MOUNTAIN WEST REGIONAL RESIDENT AWARD WINNER: ‘TENSION HEADACHE’ WITH A SURPRISING
ETIOLOGY—A CASE OF NEUROCYSTICERCOSIS
M.C. Kruer
1; P. Radhakrishnan2. 1University of Arizona, Tucson, AZ; 2St Joseph Hospital, Phoenix,
AZ. (Tracking ID #116970)
LEARNING OBJECTIVES
1. Recognize the distinctive clinical and neuroradiologic features of neurocysticercosis
(NC). 2. Recognize that eosinophilic meningitis may be caused by NC.
CASE
A 28 year old previously healthy Hispanic male presented with a history of gradual
onset bitemporal headache. It was pulsating, waxing and waning in character. During
initial assessment, he had a normal physical exam. He was instructed to take analgesics.
The patient subsequently returned with worsening left-sided fronto-temporal headache,
nausea, vomiting, fever, photophobia, back pain. His wife reported recent irritability
and emotional lability. There was no history of any recent illness, sick contacts
or trauma. The patient had immigrated to the U.S. five years ago. He was born in Mexico.
On examination, the patient appeared uncomfortable. The rest of his examination, including
fundal and neurological examinations were normal. Laboratory studies—CBC and electrolytes
were normal. CSF Protein 76 mg/dl, Glucose 40 mg/dl, WBC 267 cells/mm3, predominantly
lymphocytes, eosinophils and foamy macrophages. CT scan showed numerous, bilateral
calcified lesions in the brain parenchyma (figure 1). A subsequent MRI showed multiple
cysts in various stages of evolution, including a racemose cyst within the right Sylvian
fissure, and several active and involuting cysts, notably in the frontal temperocortex.
(figures 2&3). He was treated with Prednisolone and Albendazole.
DISCUSSION
This case is interesting as it follows the evolutionary stages of cysticercosis of
the brain. The clinical presentation of neurocysticercosis varies with the number,
location, and status of the cysticerci. Intact cysts tend to be asymptomatic, but
all cysts tend to degenerate over time. Symptoms are commonly due to the associated
immune response that occurs. In addition, completely degenerate, calcified cysts can
serve as epileptic foci. In our patient, the fronto-temporal headache was the primary
clinical manifestation, and may have resulted from involuting cysts in that region.
The eosinophilia seen in the CSF strongly supports our theory that it was the immunologic
response to the involuting cysts present on the surface of the brain that caused the
meningoencephalitis and sudden deterioration in the patient's condition. To summarize,
in patients originating from areas endemic for T. solium infection presenting with
worsening headache and eosinophilic meningitis, neurocysticercosis should be strongly
considered.
MUSIC TO THE EARS? NOT!
Y. Ng
1; R.C. Brooks2. 1University of Pittsburgh, Pittsburgh, PA; 2Pittsburgh VA Healthcare
System, Pittsburgh, PA. (Tracking ID #115803)
LEARNING OBJECTIVES
1) To identify the different categories of tinnitus and their clinical significance.
2) To determine the appropriate work up for a patient with new onset tinnitus. 3)
To recognize calcium channel blockers as a cause of drug-induced tinnitus.
CASE
Mr. L is a 60 year old man with a history of hypertension, neurofibromatosis and TIA,
who presented with new onset tinnitus of 4 weeks duration. It was a constant ringing
in both ears, but had minimally effected his life. He did not recall any recent trauma,
although he did admit to a noisy work environment. There were no other new or associated
symptoms, including no change in hearing acuity. Physical exam was only notable for
many cutaneous neurofibromas and axillary freckling. Otologic and neurologic exams
were normal. An audiologic evaluation revealed mild, bilateral high frequency hearing
loss with normal (100%) speech discrimination. MRI of the brain was normal. Nifedipine,
which was the only new medication initiated prior to the onset of his symptom, was
discontinued. During his follow up visit a month later, the patient reported that
his tinnitus had subsided after stopping the calcium channel blocker.
DISCUSSION
Tinnitus is a common complaint in ambulatory medicine. A careful, focused history
and physical examination will dictate further workup. When taking a history, tinnitus
should be categorized as pulsatile or non-pulsatile. Pulsatile tinnitus points to
an underlying vascular malformation, which may be potentially life-threatening. It
is frequently audible to the third party during examination and hence exam should
be focused on 1) auscultation for bruits over the neck, periauricular, temple and
orbital region, 2) otoscopic exam to look for vascular lesions. CT scan is the first
step in the investigation of pulsatile tinnitus; if negative, angiography will be
the gold standard. 95% of patients however, will have non-pulsatile tinnitus. Non-pulsatile
tinnitus is usually subjective and originates in the auditory system. Associated symptoms,
like hearing loss and vertigo, and precipitating factors, including medications, should
be sought. A thorough neurologic exam is important in this category. Audiometric testing
to assess for hearing loss is the best initial study. Unilateral hearing loss coupled
with poor speech discrimination is highly suggestive of a tumor; if present, MRI is
the imaging of choice, with nearly 100% sensitivity and specificity. Only 5% of hearing
tests will be normal in patients with acoustic neuroma, so initial audiometric testing
can reduce unnecessary use of MRIs. Most patients with non-pulsatile tinnitus will
have a negative work-up for a tumor. However, a large number of commonly used medications
can cause or exacerbate the symptom and discontinuation often provides significant
relief, even if the symptom does not completely resolve.
MYCOPLASMA PNEUMONIAE AND SEPSIS SYNDROME
N.D. Hare
1; K. Kieffer1. 1Dartmouth Hitchcock Medical Center, lebanon, NH. (Tracking ID #117449)
LEARNING OBJECTIVES
Recognize the diverse presentations of Mycoplasma pneumoniae infections. Diagnose
and treat Mycoplasma pneumoniae infections.
CASE
A 23 year-old white male presented with three days of malaise, fever, chills, headache,
odynophagia, lymphadenopathy, non-productive cough, myalgias, watery stools, and rash.
He was previously healthy, took no medications and had no allergies. Family history
was non-contributory. He was single, heterosexual, smoked 1 ppd cigarettes, rarely
drank alcohol, and used IV drugs 8 years previously. Vital signs on admission were:
T 38.0°C, BP 80/40, HR 120, RR 20, and oxygen saturation 94% on 2 L/min. Physical
exam revealed an erythematous pharynx, tender cervical adenopathy, clear lungs, and
a total-body macular rash. Laboratory results included: WBC 19,200 (39% bands); platelets
108,000; creatinine 1.5; CPK 453, and troponin T 0.29 (normal <0.03). A rapid streptococcal
screen and a Monospot test were negative. Chest X-ray showed a widened mediastinum.
Neck and chest CT scan showed cervical and mediastinal adenopathy. EKG showed sinus
tachycardia without ischemic changes. An echocardiogram revealed ejection fraction
20%, global biventricular hypokinesis, and no valvular vegetations. He was treated
with clindamycin and moxifloxacin, intravenous fluids and pressors. He continued to
have fevers to 40.7°C, but cultures of blood, sputum, urine, and CSF, as well as serum
and CSF viral studies, failed to reveal a source. An HIV test was negative. On hospital
day #14, serology for Mycoplasma pneumoniae IgM returned positive. Moxifloxacin had
been stopped on hospital day 7, but was resumed for a total course of 12 days. The
patient defervesced, and a repeat echocardiogram on hospital day #23 showed normalization
of ventricular function, with ejection fraction of 60%. Despite several complications,
he recovered fully and was discharged to home.
DISCUSSION
Mycoplasma pneumoniae infections are usually mild and self-limiting. The most common
target is the respiratory tract. However, M. pneumoniae can rarely cause severe infections,
affecting multiple organ systems. Besides the lungs, the skin and heart are most commonly
involved. M. pneumoniae has been documented to cause exanthems, arrhythmias, myocarditis,
neurologic problems (e.g. Guillain-Barré), and Stevens-Johnson syndrome. M. pneumoniae
lacks a cell wall, so it is not seen on Gram staining. It is not easily cultured.
Confirmation of infection is by enzyme-linked immunoassay for IgM and IgG directed
against M. pneumoniae. Due to the lack of a cell wall, Mycoplasmas are resistant to
penicillins, cephalosporins, and vancomycin. Treatment with a macrolide, a fluoroquinolone,
or doxycycline is recommended This patient presented with sepsis syndrome, which was
ultimately attributed to Mycoplasma pneumoniae and resolved with appropriate antimicrobial
therapy.
MYELITIS FROM MOSQUITOS
D. King
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117480)
LEARNING OBJECTIVES
1. Recognize the association of West Nile Virus (WNV) with acute febrile progressive
paralysis. 2. Consider viral encephalitis in the differential for immunocompromised
patients who present with neurological dysfunction.
CASE
A 43-year-old man with HIV (CD4 130) presented with five days of back pain, diarrhea
and bilateral calf cramping. He was an obese, febrile man with symmetrical 3/5 muscle
strength in upper and lower extremities. His cranial nerves and mental status were
normal. Within 48 hours his weakness progressed to 0/5 proximal muscle strength, 1/5
distal muscle strength, and 2/5 hand grip and plantar-flexion. He developed areflexia,
bilateral hearing loss and respiratory failure. A lumbar puncture was attempted but
was unsuccessful. The patient's obesity prevented an MRI. A CT scan of the head showed
only sinusitis. He was empirically treated for bacterial meningitis versus Guillian-Barre
syndrome (GBS). Encephalitis panels and serological screening for CNS infections were
drawn. The patient was discovered to have West Nile IgM; all other serological tests
were negative. EMG was performed and showed a mixed neuropathic/myopathic picture,
that, when taken into account with his hearing loss, effectively excluded GBS.
DISCUSSION
West Nile virus (WNV) is a flavivirus transmitted by mosquitos. The most common presentation
is a febrile illness and meningoencephalitis. Less common, though still prevalent,
is a syndrome of acute progressive febrile paralysis similar to poliomyelitis. Proximal
muscle weakness predominates over distal, and cranial nerve involvement is expected.
Although bacterial and fungal meningitides are the most commonly considered causes
of acute-onset neurological changes in immunocompromised individuals, viral encephalitides
should also be considered, especially in areas where mosquitos and other insect vectors
are prevalent. Physicians should recognize the poliomyelitis-like syndrome associated
with the West Nile Virus, especially in the immunocomprised.
MYOCARDIAL INFARCTION IN A YOUNG ADULT WITH ELEVATED APTT
A. Kalyanasundaram
1; A. Quiery1; K. Gavlick1. 1Geisinger Medical Center, Danville, PA. (Tracking ID
#116911)
LEARNING OBJECTIVES
1) Recognize antiphospholipid syndrome as a rare cause of myocardial infarction in
the young adult 2) Reinforce that antiphospholipid syndrome is a procoagulant disorder
despite the elevated APTT 3) Management of a myocardial infarction in the setting
of antiphospholipid syndrome.
CASE
42-year-old woman presented at an outside hospital with shortness of breath and stuttering
chest pain for one week. She was admitted in the same hospital less than a month earlier
for dysfunctional uterine bleeding when she was diagnosed with lupus anticoagulant.
Her history is otherwise unremarkable. She was transferred to our hospital with the
diagnosis of an acute MI based on EKG changes and enzyme elevation. She was tachycardic,
hypotensive and hypoxic. Chest exam revealed rales at both bases. Her PTT was elevated
at 75. Medical management was optimized and she was started on heparin after an emergent
hematology consult. An echocardiogram revealed evidence of a large inferior, inferolateral
and anterolateral myocardial infarction with left ventricular ejection fraction of
30–35%. Cardiac enzymes and Troponin-T were positive. Her dilute Russell Viper Venom
Time (dRVV) was positive (150 seconds). Her anticardiolipin antibody IgG was strongly
positive (94.7 GPL U/ml) and IgM (56.2 GPL U/ml) was medium positive. The elevated
aPTT failed to correct after mixing studies. Her heparin was titrated with serial
thromboelastograms. Cardiac catheterization revealed critical ostial left main coronary
artery disease that lead to CABG. Coumadin therapy was initiated and she was discharged
in a satisfactory condition. Repeat anticardiolipin antibodies at 6 weeks remained
elevated.
DISCUSSION
Primary antiphospholipid syndrome, also known as Hughes syndrome, is a thrombotic
disorder characterized by antiphospholipid antibodies—anticardiolipin (aCL) antibodies
and lupus anticoagulant. This syndrome presenting as coronary artery disease has rarely
been reported in the literature. Antiphospholipid syndrome resulting in a myocardial
infarction with critical ostial left main artery disease necessitating CABG has never
been reported to the best of our knowledge. ACL antibodies are strongly associated
with venous and arterial thrombosis, both in patients with systemic lupus erythematosus
and in the primary antiphospholipid syndrome. Antiphospholipid syndrome should be
considered in the differential diagnosis of myocardial infarction in the young adult.
It is important to be cognizant of the fact that despite the elevated aPTT, antiphospholipid
syndrome is a hypercoagulable state requiring anticoagulation when appropriate. Also,
thromboelastogram, a global test of coagulation, has a possible role in the management
of patients on intravenous heparin.
NEISSERIA SICCA: SO CLOSE TO MY HEART
C. Burgdorf
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117400)
LEARNING OBJECTIVES
1. Recognize N. Sicca as a cause of endocarditis in HIV-positive patients 2. Recognize
the clinical risk factors for developing N. Sicca endocarditis.
CASE
A 43 year-old man presented with a three-day history of fever and myalgias. He noted
the absence of headache, nausea, vomiting, weight loss, cough, or diarrhea. His past
medical history was notable for hepatitis C and HIV. He routinely used intravenous
drugs, but did not smoke or drink. He had no history of opportunistic infections;
his CD4 count was 344 cells/mm3 three months prior. His temperature was 38.4 °C; his
pulse was 109 beats/min. He had a 2/6 systolic murmur at the apex that increased with
hand grip. The remainder of his exam was normal. A diagnosis of endocarditis was entertained
despite prior medical records that noted a 1/6 murmur, Blood cultures were positive
for Neiserria sicca. Although considered a contaminant, a transesophageal echocardiogram
was performed to exclude the diagnosis. This revealed vegetations on the tricuspid
valve consistent with endocarditis. He was treated with ceftriaxone for six weeks.
DISCUSSION
Although Neisseria meningitidis and Neisseria gonorrhoeae are well-known pathogens,
most of the genus Neisseria species are considered commensal in as much as they colonize
but do not cause disease. Neisseria sicca is a commensal organism that is typically
found in the throat and rarely causes disease. In the setting of immunosuppression,
however, organisms that are normally commensal in nature can initiate disease. The
modified Duke criteria includes a history of intravenous drug use as a minor criteria
for the disease. The impurities in intravenous drug injection have been shown to disrupt
the endothelial lining of normal valves, predisposing the patient to endocarditis
by allowing a site of attachment for the organism. This patient's HIV and intravenous
drug use history predisposed to infection, even to a normally commensal organism.
It is important for physicians to remember that there is a higher incidence of commensal
organisms causing pathologic disease in those that are immunocompromised.
NEUROSARCOIDOSIS INITIALLY DIAGNOSED AS MULTIPLE SCLEROSIS
M. Quate-Operacz
1; E. Warm1. 1University of Cincinnati, Cincinnati, OH. (Tracking ID #115637)
LEARNING OBJECTIVES
1. Recognize the multiple presentations of neurosarcoidosis. 2. Differentiate neurosardoidosis
from multiple sclerosis.
CASE
Differentiating between the neurologic manifestations of sarcoidosis and multiple
sclerosis (MS) can be exceedingly difficult, especially when other signs of sarcoidosis
are missing. To do so is important as the therapies and prognoses of these two diseases
are vastly different. We present a case of a patient initially diagnosed with MS who
developed classic sarcoidosis 2 years later. A 38 year old male with a purported history
of MS complained of dyspnea on exertion, cough with yellow-white sputum production
and chest pain worsening over 4 months. Three skin lesions on the upper extremities
and abdomen had appeared one month prior to presentation. Two years prior to this
he had complaints of paresthesias from the umbilicus to the toes, increasing weakness
of both lower extremities, and occasional weakness and paresthesias of the right upper
extremity. A chest x-ray was normal at this time. An MRI revealed signal abnormalities
consistent with a demyelinating process at C4–C5 and T8–T9 levels. An LP demonstrated
elevated protein and oligoclonal bands. The patient was diagnosed with MS and started
on interferon-beta therapy with resolution of symptoms. On the current presentation
the patient's chest x-ray revealed diffuse nodular infiltrates. Biopsy of the skin
lesions was consistent with sarcoidosis. In retrospect, it was concluded that the
initial presentation with paresthesias and weakness was most likely attributable to
neurosarcoidosis.
DISCUSSION
Neurosarcoidosis is an uncommon manifestation of systemic sarcoidosis. In clinical
studies, it was present in 5–16% of sarcoid cases. Neurologic complaints are the presenting
symptom of neurosarcoidosis in 50% of cases. The majority will also have disease in
other organ systems. The diagnostic criteria include a compatible clinical picture,
typical radiologic findings, and histologic evidence of sarcoid from any tissue. MRI
findings vary but white matter lesions can be present and mimic MS. Up to 80% will
have CSF abnormalities that include mononuclear pleocytosis, increased CSF pressure,
and some cases there has been evidence of oligoclonal bands making it very difficult
to distinguish from MS. Before treatment is instituted 35–50% of cases will improve
spontaneously. One third can relapse again simulating MS. This remission and relapse
are likely what occurred in our patient as studies have that shown sarcoid and other
autoimmune diseases can actually be exacerbated during interferon therapy.
NEUROSYPHILIS PRESENTING AS BILATERAL VOCAL CORD PARALYSIS AND GASTROINTESTINAL AUTONOMIC
DYSFUNCTION
D. Blenner
1; C. Woods1; R. Levy1; J.C. Byrd1. 1East Carolina University, Greenville, NC. (Tracking
ID #117338)
LEARNING OBJECTIVES
To emphasize the importance of including neurosyphilis in the differential diagnosis
of unusual or unexplained neurological findings in patients who are immunocompromised.
CASE
A 34 year old male presented to our service after sustaining head trauma from a MVC
causing a subarachnoid hemorrhage (SAH) and subsequent short-term memory deficits
and generalized weakness. On day 16 after his injury he developed stridor. Laryngoscopy
revealed bilateral vocal cord paralysis (BVCP). A head CT scan showed no acute processes.
A tracheostomy was performed to protect his airway. A percutaneous gastostomy tube
was placed to provide adequate nutrition but he had persistent residuals with his
feedings. On day 29, the patient developed a left-sided T6 dermatomal herpes zoster
rash. A thorough sexual history revealed that the patient had engaged in unprotected
sexual activities with men. STD serologies were ordered with the patient's consent.
The RPR and HIV were positive. A lumbar puncture was performed which revealed 43 white
blood cells that were predominantly lymphocytes and a positive VDRL. Treatment for
neurosyphillis was initiated with 24 million units of Penicillin G per day. After
four days of treatment, the patient's voice began to return and his feeding residuals
resolved. He was discharged one week into therapy with normal phonation. Subsequent
laryngoscopy showed near complete recovery of his vocal cords. Literature review revealed
that syphilis can be rare cause of vocal cord paralysis.
DISCUSSION
With the increasing rates of syphilis in the past 2 years and its frequent association
with HIV, it is important to recognize that neurosyphilis is not uncommon and may
present in a cryptic fashion.
NO DRUG IS BENIGN: NEUROLEPTIC MALIGNANT SYNDROME CAUSED BY AN ATYPICAL ANTI-PSYCHOTIC
V. Chan
1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116214)
LEARNING OBJECTIVES
1. Recognize clinical features of Neuroleptic Malignant Syndrome (NMS). 2. Recognize
that newer, atypical agents can still cause NMS. 3. Recognize managment options for
NMS.
CASE
A 65 year old Caucasian male with schizophrenia presented with a two days of progressive
change in mental status. According to his care givers, he was no longer performing
usual activities of daily living including eating, dressing, and bathing. He had been
found with bowel and bladder incontinence and was no longer speaking or following
commands. His medications included olanzapine 10 mg po qd and temazopam 30 mg po qhs.
On exam, he was alert, but with a masked facies. Orientation and cranial nerve function
could not be assessed. He could move all extremities spontaneously, but had diffusely
increased tone and a 3 second upper extremity resting tremor. Non-contrast head CT,
EEG and blood and urine studies were negative. He was diagnosed with neuroleptic induced
extrapyramidal symptoms. Fluids were started and olanzepine was discontinued. On hospital
day #3, he developed a fever to 100.6 F and was acutely confused. He had pulse 128,
blood pressure 120–160 systolic. He had lead pipe muscular rigidity, increased tone
compared to admission, and diffusely brisk deep tendon reflexes. Lab data revealed
CPK = 2,890 U/L, Na = 154. CBC, chemistries, blood/urine cultures and CXR were negative.
Brain MRI showed chronic small vessel ischemic changes, but no acute pathology. The
patient was diagnosed with olanzapine induced neuroleptic malignant syndrome. He responded
nicely to treatment with dantrolene, bromocriptene, and intravenous fluids.
DISCUSSION
Neuroleptic Malignant Syndrome (NMS), an idiosyncratic reaction to antipsychotic agents,
is assumed to be due to reduced dopaminergic activity. It is characterized by severe
rigidity, tremor, altered mental status, fever, and autonomic dysfunction. Complications
include rhabdomyolyis, acute renal failure, and thromboembolism. It is traditionally
associated with older antipsychotics (haloperidol, risperidone), but there have been
some case reports with newer agents such as olanzepine. Risk factors include dehydration,
poorly controlled neuroleptic induced extrapyramidal symptoms (EPS), treatment resistant
EPS, and rapid rate of neuroleptic loading. Treatment involves discontinuation of
neuroleptics and supportive care with antipyretics, fluids, and electrolytes. Refractory
cases require initiation of dopamine agonists and muscle relaxants, such as bromocriptine
and dantrolene. Severe NMS has 20–30% mortality. Our case underscores the importance
for generalists to recognize the side effect profile of medications prescribed by
other specialties. It also demonstrates that newer agents can still cause NMS.
NONTUBERCULOUS MYCOBACTERIUM AS A CAUSE OF BURSITIS
H.E. Woo
1; C.H. Fung2. 1University of California, Los Angeles, Los Angeles, CA; 2VA Greater
Los Angeles Healthcare System, Los Angeles, CA. (Tracking ID #116278)
LEARNING OBJECTIVES
1. Recognize nontuberculous mycobacterium as a cause of bursitis. 2. Manage Mycobacterium
chelonae infection with consultation from infectious diseases and surgery.
CASE
A 63-year old retired probation officer and former alcoholic presented with a chief
complaint of one month of right elbow pain and swelling. He had been performing physical
therapy floor exercises, which included weight-bearing stances on both elbows, for
chronic back pain. His favorite location to practice the exercises was at the local
beach in southern California. Physical exam revealed a well-developed, well-nourished
male in no acute distress. He was afebrile and had a 5 × 5 cm fluctuant, warm, tender,
erythematous swelling over the right olecranon without open wound or overlying rash.
Fine needle aspiration was performed during the initial clinic visit. Culture of the
aspirated bursa fluid grew Mycobacterium chelonae, sensitive only to clarithromycin
and aminoglycosides. He started oral clarithromycin and had such a dramatic response
that the patient refused recommended treatment with intravenous (IV) amikacin and
bursectomy. He agreed to discontinue physical therapy exercises involving elbow weight-bearing
stances. Seventeen months later he presented with pain and swelling involving the
opposite (left) olecranon bursa; the right olecranon was normal on examination. Aspiration
again showed Mycobacterium chelonae sensitive only to clarithromycin and aminoglycosides.
With consultation from infectious diseases and orthopedics, he had a successful left
bursectomy while receiving a six-week course of oral clarithromycin and two-week outpatient
course of IV amikacin via percutaneous intravenous central catheter (PICC line).
DISCUSSION
M. chelonae is a nontuberculous mycobacterium with worldwide distribution that can
be found in natural and processed water sources such as tap water and sewage. Nosocomial
infections can occur as a result of instruments contaminated with colonized tap water.
Presentation varies according to the site, but superficial infections typically are
nonhealing, nonspreading wounds. Diagnosis requires smear for acid-fast bacilli and
culture. Unlike tuberculosis, M. chelonae infections do not need to be reported to
local health departments. Management with a surgeon is indicated, because without
surgical debridement recurrence is likely. Consultation with infectious disease specialists
is appropriate for therapeutic guidance. M. chelonae is resistant to typical antituberculous
medications such as rifampin and isoniazid. However, it is usually sensitive to clarithromycin
and amikacin. Resistance to single-drug therapy occurs, so dual antibiotic therapy
is typically recommended. The optimal duration of antibiotic therapy is unknown, but
ranges from weeks to months.
NOT JUST ANOTHER HEART FAILURE (HF) EXACERBATION: SIGNIFICANT VOLUME OVERLOAD FROM
A THIAZOLIDINEDIONE (TZD)
R.M. Malone
1; M.P. Pignone1; A.B. Weil1; B. Bryant1. 1University of North Carolina at Chapel
Hill, Chapel Hill, NC. (Tracking ID #115896)
LEARNING OBJECTIVES
1) Recognize the potential of TZDs to potentiate or exacerbate symptoms of HF; 2)
Discuss proper use of TZDs in light of the potential adverse effects; 3) Identify
patient education and counseling as a way to avoid complications from volume overload
when TZDs are used.
CASE
JN is a 59 year old woman with type 2 diabetes, chronic renal insufficiency, and HF
from viral myocarditis in 1991. An echocardiogram from February 2000 revealed a LVEF
of 32%. She presented to clinic July 2001 with increased fatigue, decreased exercise
tolerance for 3 months, DOE, and edema. She denied SOB at rest, PND, or orthopnea.
Functional status had worsened from NYHA class II to III. Medications included: Pioglitazone
45 mg qd (Added 4/2001), Digoxin 0.0625 mg qd, Furosemide 80 mg qam 40 mg qpm, Lisinopril
20 mg qd, Metoprolol XL 50 mg qd, Warfarin 5 mg qd, Simvastatin 20 mg qd, Spironolactone
25 mg bid, NPH insulin 20u am and 10u pm, REG insulin 10u bid. Vitals: 271 lbs (15
lb increase over the preceding 2 months), BP 90/70, P 88. Physical Exam: 15 cm of
JVD, displaced PMI, distant S1 and S2, 2–3+ bilateral lower extremity peripheral edema.
Recent labs: SCr 1.6, BUN 59, ALT 27, and A1c 7% (previous A1c 11.1%). Furosemide
was increased to 80 mg bid and genotyping for cardiac transplantation was ordered.
Over the following 3 months, JN had 6 cardiology and medicine clinic visits and continued
to complain of symptoms of DOE, edema, and an eventual weight gain of 30 lbs. She
was hospitalized October 2001 for intravenous diuresis. After discharge, the General
Medicine Diabetes Program decreased Pioglitazone to 30 mg over the telephone. This
resulted in improved symptoms and a 28 lb weight loss over the following month. The
Diabetes Program discontinued the TZD December 2001, her weight returned to baseline
and functional status improved to NYHA class II.
DISCUSSION
Pioglitazone and Rosiglitazone are TZD antidiabetic agents that activate PPAR-gamma
thus reducing insulin resistance. When used as monotherapy, TZDs can decrease A1c
approximately 1.5%. TZD package inserts list HF, dose related edema, and weight gain
as potential adverse effects. Average weight gain ranges from 0.8 to 5.4 kg and edema
occurs in 4.8 to 15% of patients. In a recent cohort study, the incidence of HF symptoms
and hospitalization for HF was 8.8% and 2.5%, respectively. Volume related adverse
events occur frequently, are often misdiagnosed, and may lead to significant patient
morbidity. Providers should recognize these potential adverse effects and avoid TZDs
in patients with or at risk for HF. Careful monitoring for weight gain, edema, and
HF symptoms is crucial with TZD use.
NOT JUST ANOTHER RASH
A. Harzstark
1; P.P. Balingit2. 1University of California, Los Angeles, Los Angeles, CA; 2UCLA
San Fernando Valley Program, Sylmar, CA. (Tracking ID #117547)
LEARNING OBJECTIVES
1. Recognize epidermodysplasia verruciformis (EV) as a predisposing factor for cutaneous
squamous cell malignancies. 2. Describe the etiology and presentation of EV.
CASE
A 21 year old Honduran female with no significant past medical history presented to
an urgent care clinic with a painless, nonpruritic, scaly lesion with associated eschar
in the right forehead area. The lesion bled intermittently from the margins and slowly
increased in size during the three years prior to presentation. The patient also reported
the eruption of generalized, erythematous plaques over her back, trunk, and extremities
starting at the age of eight. These skin lesions were also present in three paternal
cousins. Physical examination was notable for a well-defined two by two centimeter
eschar on the patient's right forehead with slight bleeding from the lateral margin.
Lesions were present, most notable on her neck, upper chest, and axillae, and also
involved her abdomen, back, arms, and legs. The rash consisted of flattened, erythematous
and hyperpigmented papules with a scaly surface and irregular borders. Areas of confluent
plaques were present as well. Biopsy of the forehead lesion showed squamous cell carcinoma
in-situ with adjacent actinic keratosis. Punch biopsy of a lesion on the back revealed
slight hyperkeratosis and irregular mild acanthosis with scattered areas of atypical
keratinocytes in the upper half of the epidermis, consistent with EV. Plastic surgery
consultation was obtained for excision of the lesion located on the patient's forehead.
Additionally, routine primary care follow-up was recommended for periodic examination
of the patient's skin to identify possible developing malignancies.
DISCUSSION
EV is an autosomal recessive disorder of the skin associated with chronic human papillomavirus
(HPV) infection. Rare cases of autosomal dominant and X-linked transmission have also
occurred. EV is associated with impaired cellular immunity to HPV, making patients
susceptible to widespread viral infection. Resulting skin lesions transform to skin
cancers, particularly in sun-exposed areas, in one-third of patients and usually after
the age of thirty. EV typically presents during childhood but can also appear during
infancy and adolescence. On histologic examination, more than 90% of EV skin lesions
contain evidence of infection with HPV subtypes 5, 8, and 47. Excision is required
for EV lesions which have transformed into malignant skin cancers. Topical retinoid
and intralesional interferon treatments for growing EV lesions have been found to
be of possible benefit in slowing the progression to skin cancer. By providing periodic
examination of the skin and surveillance of lesions, the primary care physician plays
a vital role in the management of EV. Biopsy of enlarging lesions is necessary to
exclude malignant transformation.
ONE FLU OVER THE CUCKOO's NEST
H.T. Ly
1; M. Rotblatt2. 1University of California, Los Angeles, Sylmar, CA; 2UCLA SFVP-Olive
View Medical Center Department of Internal Medicine, Sylmar, CA. (Tracking ID #115582)
LEARNING OBJECTIVES
1. To recognize the severity of complications and the impact of influenza illness
2. To ultimately realize the importance of the flu vaccine.
CASE
A 50 year old saxophone player presented to the ED with a week of “chest cold,” cough
productive of yellowish sputum, intermittent fevers as high as 102 F, and nightsweats.
He was a smoker but had no significant PMH. He had no recent travel or risk factors
for tuberculosis, although he had been visiting his ill wife in the hospital in recent
weeks. She was a surgical patient whose hospital course was complicated by pneumonia.
His vital signs were critical: T 38.3 C, BP 71/52, P 150, R 28, Pulse Ox 84% on 2L
nasal cannula. There were diffuse crackles and tachycardia on exam. His WBC was 7.0
(N 79.6, L 9.9, M 10.4), and his BUN/Cr were 36/2.2. His CXR showed large bibasilar
infiltrates. He was admitted to the ICU with a diagnosis of severe community acquired
pneumonia and sepsis. He was intubated and received piperacillin/tazobactam, azithromycin,
and aggressive fluid hydration. Sputum and blood cultures were sent, and the patient
was stabilized but remained febrile. On day 2, both sputum and blood cultures were
overwhelmingly positive for methicillin resistant Staphylococcus aureus (MRSA). Vancomycin
was started, and he defervesced within 2 days. His PPD, sputum PCP DFA, AFB smears,
and fungal cultures were negative. However, his pulmonary status remained poor, prompting
a chest CT that showed extensive bilateral bronchiectasis and cavitation that was
attributed to the Staph infection. His hospital course was long, eventually requiring
transfer to a ventilation weaning facility. Interestingly, he was initially tested
and found positive for influenza A. He had not received the flu vaccine that season.
DISCUSSION
Our patient likely had influenza initially, which predisposed his lungs to a secondary
bacterial infection. Pneumonia, especially with Staph, is a classic complication of
influenza. Acquiring an MRSA infection, however, was a curiosity and eventually was
attributed to his recent visits to the hospital to visit his ill wife. This case highlights
a serious course that followed a seemingly commonplace flu. Given the severity of
complications and dramatic rise in influenza cases this season, it becomes paramount
to realize the impact of the flu and the potential protection offered by the vaccine
to the general public.
PARALYSIS IN A YOUNG ADULT: AN UNUSUAL DIAGNOSIS
D. Fotino
1; M. Landry1. 1Tulane Health Sciences Center, New Orleans, LA. (Tracking ID #117459)
LEARNING OBJECTIVES
1) Recognition of causes of macrocytic anemia 2) Identify diagnosis and treatment
of pernicious anemia 3) Develop a diagnosis in ascending progressive neurologic deficits.
CASE
A 39 year-old woman with diabetes mellitus presented with inability to walk. Her symptoms
began eight months prior with gradual progression leading to inability to ambulate.
She noted decreased bilateral lower extremity proprioception with progressive ascending
sensorimotor loss. She reported decreased appetite, weight loss, dysphagia, incontinence
and worsening back pain. Physical examination revealed thyromegaly, sacral decubiti
and lower extremity edema. The neurologic examination was remarkable for short-term
memory loss. Her lower extremity examination revealed sensory deficits to light touch
and pin-prick, absent reflexes and motor paralysis. Rectal tone was poor. Diagnostic
studies included a hemoglobin of 8.8 g/dl with an MCV 112 Ym£, B12 level <100, iron
saturation 6% and normal thyroid studies. Electromyogram revealed diffuse axonal and
demyelinating loss in her lower extremities. Her esophagogastroduodenoscopy demonstrated
atrophic gastritis and her anti-intrinsic factor antibody was positive. She underwent
B12 replacement with normalization of her MCV, but her neurologic deficiencies worsened.
A repeat EMG was consistent with Amyotrophic Lateral Sclerosis (ALS).
DISCUSSION
Common causes of macrocytic anemia include folate and B12 deficiency, and liver and
thyroid disease. Initial evaluations include folate, B12, liver and thyroid studies.
Pernicious anemia causes B12 deficiency with antibodies against intrinsic factor and
parietal cells. Neurologic sequelae of B12 deficiency may be reversed with B12 replacement
and normalization of the MCV. Further evaluation may be necessary for progressive
neurologic symptoms. ALS is a severe form of progressive degenerative neurologic disease
and must be considered in the differential of worsening neurologic deficits. Patients
with ALS often become sufficiently malnourished to lead to concomitant vitamin deficiencies.
The failure to respond to parenteral B12 was an important clue in investigating ALS
as an etiology for her neurologic deficits.
PARATHYROID CARCINOMA: TWO CASES DIAGNOSED PRE OPERATIVELY
K. Pachipala
1; S. Naidu1; D. Bucaloiu1; R. Pierce1; R. Monsaert1. 1Geisinger Medical Center, Danville,
PA. (Tracking ID #109732)
LEARNING OBJECTIVES
Recognize that parathyroid carcinoma can be clinically suspected preoperatively.
CASE
Case 1 A 66-year-old man presented with a 5-month history of pain in his right heel.
Serum calcium and iPTH were elevated at 12.6 and 1800 respectively. Skull x-ray showed
a salt and pepper appearance and a large “brown tumor”. A bone scan showed increased
uptake in the heel and skull. Because of significant bone involvement and a markedly
elevated PTH, parathyroid carcinoma was suspected. A large left inferior parathyroid
gland was found and resected. Microscopic examination confirmed parathyroid carcinoma.
The patient developed the “hungry bone syndrome” following surgery, and responded
to calcium and vitamin D. Case 2 A 55-year-old woman with a history of symptoms typical
of hypercalcemia came to attention when a biopsy of bone from a pathologic fracture
of her patella revealed a “brown tumor”. Serum calcium and iPTH were 18.4 and 1120
respectively. Because of severe hypercalcemia, bone involvement and a markedly elevated
PTH, parathyroid carcinoma was suspected. A totally intrathyroidal parathyroid carcinoma
was resected. She also developed the “hungry bone syndrome”.
DISCUSSION
Hyperparathyroidism-dependent hypercalcemia is commonly due to parathyroid hyperplasia
or an adenoma. Parathyroid carcinoma is an uncommon cause with 390 cases reported
so far in the literature. Parathyroid carcinoma is an indolent tumor and the clinical
features are predominantly due to the effects of excess PTH secretion. It is important
to suspect parathyroid carcinoma preoperatively, as a complete resection of the tumor
at the time of initial operation is required for an optimal outcome. Often, unfortunately
the diagnosis is made when hypercalcemia recurs after surgical treatment of primary
hyperparathyroidism. Parathyroid carcinoma may be suspected clinically by the presence
of severe and symptomatic hypercalcemia, markedly elevated PTH levels (3–10 times),
palpable neck mass and a high prevalence of renal and skeletal involvement. Our high
suspicion for parathyroid carcinoma alerted the surgeon to do a more aggressive and
potentially curative, surgical procedure. Significant hypocalcemia from hungry bone
syndrome is common postoperatively and is regarded as a sign that the surgery is successful.
PARATHYROID STORM
E.H. Orth
1; S. Mangers1; L. Cation1. 1University of Illinois at Peoria, Peoria, IL. (Tracking
ID #116240)
LEARNING OBJECTIVES
Recognize the clinical features of severe hypercalcemia. Recognize benign primary
hyperparathyroidism can not be excluded in the differential of severe hypercalcemia.
CASE
A 48 year old female presented with a 5 day history of progressive weakness. She complained
of anorexia, vomiting, abdominal pain, as well as constipation and polyuria. Further
history included headache, difficulty thinking, cough, and dysphagia. On physical
exam, she was alert but displayed slow and labored conversation. The exam was remarkable
for dehydration, tachypnea, and fullness of the right neck without discernible lesion.
The abdomen had decreased bowel sounds and was soft with moderately diffuse tenderness.
There was no guarding or rebound. The remainder of the exam was unremarkable. Laboratory
analysis revealed serum calcium 23.6 mg/dl, phosphate 2.2 mg/dl, intact parathyroid
hormone (iPTH) 1435 pg/ml, and serum electrophoresis with polyclonal increase in gamma
globulin. The chest radiograph was unremarkable. A limited parathyroid scan had increased
activity throughout an elongated right thyroid lobe. The patient was rehydrated, then
given furosemide and pamidronate. Serum calcium fell to 11.3 mg/dl. Parathyroid surgery
with neck exploration was performed and an 18.21gram mass was removed from the right
inferior parathyroid gland. Pathologic examination revealed a parathyroid adenoma
with mild nuclear atypia, low mitotic activity, and no malignancy. One day post surgery,
serum calcium was 9.6 mg/dl. The patient had clinical resolution of her signs and
symptoms.
DISCUSSION
Most patients with primary hyperparathyroidism are asymptomatic with only mild elevation
of serum calcium. Extreme calcium level elevations are more typical of parathyroid
malignancy. Upon literature review, the highest reported serum calcium in benign PHPTH
was 26.3 mg/dl in 1987. We report a case of a symptomatic patient with serum calcium
23.6 mg/dl and iPTH 1435 pg/ml resulting from a benign parathyroid adenoma. Benign
primary hyperparathyroidism can not be excluded in the differential of severe hypercalcemia.
PARTIAL SPINAL CORD SYNDROME IN A PATIENT WITH NASOPHARYNGEAL CARCINOMA
R. Bomprezzi
1; P. Radhakrishnan2. 1St. Joseph Hospital, Phoenix, AZ; 2Catholic Healthcare West,
Phoenix, AZ. (Tracking ID #116023)
LEARNING OBJECTIVES
1. Recognize the rare neurological manifestations of invasive neck tumors and the
treatment. 2. Diagnose partial spinal cord syndromes. 3. Recognize that autonomic
dysfunction should be considered in the differential diagnosis of patients with bradycardia
and hypotension.
CASE
A 77 year-old male patient presented with a history of syncope. He was found unresponsive
for an unknown duration. His past medical history included nasopharyngeal carcinoma
diagnosed four years before, for which he underwent extensive surgery to the right
side of his neck, including laminectomy at the level of C3–C4 and subsequent radiation
therapy. He also had Rheumatic Heart Disease with moderate mitral and aortic regurgitation
and hypertrophic cardiomyopathy . Physical Exam—He was alert and oriented. BP 85/50
mmHg, HR 40/min. Pupils were equal and reactive bilaterally. Neck exam. revealed a
left submandibular mass. There was swelling of the left base of the tongue with mild
dysarthria. CVS-apical systolic murmur with no radiation. Neurological exam-decreased
sensation in the left half of the face. Sensation to temperature was decreased on
the left side of the body, more pronounced on the lower extremity; proprioceptive
and tactile sensations were preserved. Motor examination revealed moderate impairment
to fine movements of right hand and decreased strength (grade 3/5) of the right leg.
The rest of the exam was normal.
DISCUSSION
This case highlights the neurologic sequele of invasive neck tumors. The Brown-Sequard
syndrome is due to hemisection of the spinal cord, usually due to trauma or spinal
cord tumors. There is ipsilateral motor weakness, proprioceptive and vibratory loss,
and contralateral loss of pain and temperature sensation. The neurological findings
described in this case are consistent with partial (incomplete) Brown-Sequard syndrome.
Injury to the descending fibers of the corticospinal tract leads to the ipsilateral
motor impairment. Damage to the ascending decussated fibers of the spinothalamic tract
accounts for the loss thermal sensation in the contralateral body. The left facial
hypoesthesia and dysarthria was due to local invasion of the recurrent tumor. The
bradycardia and hypotension in this patient could be due to either autonomic dysfunction
secondary to the neck mass or the patient's underlying cardiac disease. To summarize,
it is important for physicians to familiarize themselves with the different clinical
manifestations of neck masses and surgery.
PATIENCE IS A VIRTUE: A SEVERE LAB ABNORMALITY WITH CAUTIOUS MANAGEMENT
E. Lee
1; L. Thomas1; H. Jasti1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID
#115958)
LEARNING OBJECTIVES
1. To identify the multi-factorial etiologies of hyponatremia 2. To recognize the
importance of history and clinical presentation in hyponatremia.
CASE
A 71 year old woman with a PMH significant for hypothyroidism, atrial fibrillation,
and mild mental retardation presented with generalized weakness, bilateral lower extremity
swelling, and “aphasia” for several weeks. On presentation, she was able follow all
commands and communicate non-verbally but with an extremely blunted affect. Physical
exam revealed mild jugular venous distension, an irregularly irregular heart beat,
moderate rales in the lung bases, and 2+ pitting edema of the legs bilaterally. MRI/MRA
of the head was normal. Chest radiograph showed pulmonary edema, a right-sided solitary
pulmonary nodule, and a small pleural effusion on the same side. Laboratory results
were significant for an elevated beta-natriuretic peptide (BNP) of 241 pg/ml, normal
TSH and cortisol levels, and a sodium of 103 mEq/L. Diagnostic thoracentesis revealed
a transudative fluid, negative for infection or malignancy. Additional history later
obtained from the family revealed that the patient had a decreased appetite over the
last 2–3 weeks, but had continued to ingest large quantities of water. At baseline,
she had a blunted affect and recently had been started on paroxetine for depression.
The drug was discontinued and the patient was placed on water restriction. The sodium
levels gradually normalized over the next five days with resolution of her symptoms.
DISCUSSION
Hyponatremia is a common lab abnormality that reflects an underlying problem with
free water balance in the body. This balance can be upset in states of excess free
water, either through impaired excretion or excess intake. In this patient, multiple
factors potentially affected her free water balance: 1) excess free water intake,
also known as primary polydipsia; 2) CHF with volume overload; 3) inadequate dietary
solute intake, or the “tea and toast diet;” 4) the syndrome of inappropriate anti-diuretic
hormone (SIADH) associated with pulmonary processes, such as malignancy; and 5) SIADH
that has been associated with paroxetine, a selective serotonin reuptake inhibitor
(SSRI). This case illustrates the importance of taking a thorough history and assessing
the clinical presentation. The etiology of the patient's hyponatremia was not clear,
as she had numerous predisposing factors. It was more important to realize the gradual
nature of her impairment. Rapid correction of her sodium levels was not necessary,
and in fact may have been more harmful. A conservative approach was taken and her
hyponatremia resolved slowly over time.
PATIENT STILL HAS FEVER OF UNKNOWN ORIGIN
F. Aslam
1; A. Mirza2. 1Geisinger Medical Center Danville PA, Danville, PA; 2Geisinger Medical
Center, Danville, PA. (Tracking ID #115629)
LEARNING OBJECTIVES
1. To identify role of adult Still's Disease (ASD) in the differential diagnosis of
Fever of Unknown origin (FUO). 2. To appreciate the difficulty in diagnosing ASD due
to lack of availability of specific diagnostic test. 3. Recognize importance of marked
hyperferritinemia in association with other diagnostic criterion in diagnosing ASD.
CASE
A 40 year white male was transferred to our tertiary care medical center for evaluation
of FUO. He was extensively investigated and evaluated by many sub-specialists during
4-week stay at another hospital. On admission to our hospital his symptoms were persistent
muscle pain, lethargy and non-specific joint pain. Physical examination revealed temperature
39°C and pulse 90/minute, however no localizing signs were present. Laboratory studies
revealed White Cell Count of 25.3 K/uL (normal range 4–10.8), Erythrocyte Sedimentation
Rate (ESR) 98 mm/hr (0–15) and Alkaline Phosphates 162 U/L (25–125). He continued
to be febrile with peak temperature of up to 40°C. An extensive workup including chest
X-ray, duplex scan of lower extremities, Bone Marrow biopsy and CT scan of chest abdomen
and pelvis was unrevealing. Tests were negative for HIV-1, heterophil antibodies,
Hepatitis A, B, C viruses, IgM and IgG antibodies against Cytomegalovirus, Epstein
Barr virus, Borrelia Burgdorferi and Brucella Abortus. Serology for dsDNA, Antinuclear
antibodies and Rheumatoid factor (RF) was negative. Blood and Urine cultures were
sterile. Suspicion of ASD was raised by markedly elevated serum ferretin level to
10,154 ng/mL (30–400) and diagnosis of ASD was made on the basis of Yamaguchi and
Kahn's criterion. Patient was treated with systemic corticosteroids with resolution
of fever and symptoms within 24 hours. At 2 weeks follow-up visit patient was symptom
free, leukocytosis had resolved and serum ferritin level had decreased to 1,068 ng/mL.
DISCUSSION
FUO is due to infection in 30 to 50 percent of cases, to cancer in 25 to 30 percent,
and to autoimmune disease in 15 to 25 percent. Among autoimmune diseases ASD is one
of the commonest cause of FUO. In ASD fever, elevated ESR and negative RF are present
in 100% patients. Other features include arthralgias (90%), Leukocytosis (100%), rash
(85%) and arthritis (65%). Since no specific test is available diagnosis is based
on exclusion of other diseases and applying diagnostic criterion. For this reason
average delay for the diagnosis is 3 to 8 weeks. Acute phase reactants are characteristically
elevated. One of the features although nonspecific is extreme elevation in serum ferritin
levels. Depending on the severity of disease nonsteroidal anti-inflammatory drug,
corticosteroids, and immunomodulating drugs can be used for treatment. Neutralization
of serum ferritin level is reliable index of success of therapy.
PERNICIOUS ANEMIA WITH SPLENOMEGALY IN A YOUNG MAN
M. Pillai
1; P. Hu1; N. Le1. 1Baylor College of Medicine, Houston, TX. (Tracking ID #116881)
LEARNING OBJECTIVES
1) Review the clinical manifestations of severe B12 deficiency. 2) Review the diagnosis
and treatment of Pernicious Anemia.
CASE
A 37-year-old white man with no significant past medical history presented to our
clinic complaining of progressive fatigue. One year ago he was able to run 3 miles
daily. Upon presentation he had shortness of breath and fatigue with minimal activity.
In the past six months, he lost 40 lbs and had symptoms of abdominal pain and early
satiety. He had gone to several doctors who just prescribed him pantoprazole which
minimally alleviated his symptoms. Physical exam was significant for a smooth red
tongue, III/VI systolic flow murmur, and splenomegaly of 15 cm. There were no neurological
deficits. Laboratory results showed WBC 2.1, Hg 6.0, Hct 17.7, MCV 102.5 and Platelets
75. A peripheral blood smear displayed macroovalocytes and multilobulated neutrophils.
His B12 level was 48 pg/ml, and serum was positive for intrinsic factor antibody.
The patient was diagnosed with pernicious anemia, (PA), and treated with cyanocobalamin
injections with marked improvement.
DISCUSSION
PA typically presents later in life. Only about 10% of cases involve patients <40
years old. Typically, elderly patients present with macrocytosis with or without anemia
and multilobulated neutrophils. However, severe cases of cobalamin deficiency can
present with pancytopenia, neurological deficits involving the dorsal and lateral
spinal columns, and even splenomegaly. Prior to early detection and treatment the
reported incidence of splenomegaly in PA varied from 3% to 45%. With advances in medicine
resulting in earlier detection, it is now rare to see splenomegaly in PA. A peripheral
blood smear showing macrocytic anemia and multilobulated neutrophils is highly specific
for B12 deficiency. The diagnosis is clinched with low serum B12 levels. Today, the
use of the Schilling test has been supplanted by serologic testing for intrinsic factor
antibodies. The presence of anti-intrinsic factor antibodies is highly specific and
confirmatory for the diagnosis of PA, with a sensitivity varying from 50 to 84%, depending
upon the population tested. Treatment with parenteral or oral cobalamin is highly
effective. B12 supplementation normalizes hemoglobin levels within months and can
reverse neurological deficits as well as splenomegaly.
PICK THE DEMENTIA
A.M. Wilson1; P. Koneru1; G. Prakash1; R.D. Hobbs
1. 1Oakwood Healthcare System, Dearborn, MI. (Tracking ID #117213)
LEARNING OBJECTIVES
To recognize the clinical significance of language impairment in the diagnosis of
dementia.
CASE
A 71 year-old independently living woman with a two-year history of cognitive dysfunction
presented two weeks following a car accident. She was alert, socially active and regularly
played tennis. The MMSE was 9/31. Her spelling ability had decreased (diner is reddy),
her speech was fluid and well enunciated but complicated by word substitution errors
such as describing blowing leaves as “flies.” The remainder of the exam and the laboratory
tests were normal. An MRI showed nonspecific cortical atrophy with nondiagnostic white
matter changes. She was diagnosed with Pick's Disease.
DISCUSSION
Arnold Pick first described Pick's disease in 1892 while reporting a series of unusual
dementias. In 1911 Alois Alzheimer described the ballooning degeneration of neurons
and the eosinophilic intraneuronal inclusions that are now known as “Pick bodies.”
Unlike Alzheimer's disease with which it is frequently confused, Pick's disease is
characterized by early expressive aphasia, personality changes and prominent problems
with language such as substitution and syntactical errors. These language problems
may complicate the diagnosis since most screening tests are language based and may
lead to falsely lowered scores. Memory loss is not an early finding as it is in Alzheimer's
disease. This distinction is significant since the problem—memory or language, occurs
at different times and may affect families and caregiver expectations. It also underscores
the fact that memory loss is not necessary to make a diagnosis of dementia.
POLYMICROBIAL ENDOCARDITIS IN AN INTRAVENOUS DRUG ABUSER
R.P. Warrier
1; J.A. Chang1; J. Jarrett1; A.C. Maio1. 1Creighton University Medical Center, Omaha,
NE. (Tracking ID #116419)
LEARNING OBJECTIVES
1. Recognize that polymicrobial endocarditis occurs in about 4–6% of all endocarditis.
2. Identify risk factors for polymicrobial endocarditis. 3. Manage infective endocarditis
with surgical intervention in cases of persistent bacteremia despite maximal antimicrobial
therapy.
CASE
Patient is a 39-year-old African-American male with history of current IVDA who presented
with cough, fever, chills, and increasing dyspnea of 10 days duration. His past medical
history included Hepatitis C, hypertension, and COPD. He had been treated for Staphylococcal
bacteremia with four weeks of Nafcillin about three months ago. At that time TEE was
negative for vegetations. Blood cultures were negative at the time of discharge. On
physical examination, his temperature was 102.7 F. Vital signs were stable. He had
no peripheral stigmata of endocarditis. A soft holosystolic murmur was heard along
the left sternal border. There was no clinical evidence of heart failure. Multiple
needle tracks were visible in his skin. Initial labs revealed leukocytosis (WBC 28.4),
with 13% bands. Chest X-ray and EKG were normal at the time of admission. Blood cultures
persistently grew
Staphylococcus aureus
and
Bacillus species
. TEE was positive for 2.6 × 1.5 cm vegetation on the tricuspid valve with severe
tricuspid regurgitation. Patient was started on Vancomycin and Rifampin based on sensitivities.
Chest CT scan showed evidence of septic pulmonary emboli on the 7th day of hospitalization.
Blood cultures remained positive after two weeks of adequate therapy. He then underwent
partial tricuspid valve replacement with mitral valve homograft and valve annuloplasty.
Vegetation was positive for the same organisms. Following surgery, patient was treated
with intravenous antibiotics for four weeks and was discharged in stable condition.
DISCUSSION
Polymicrobial endocarditis is rare, about 4–6% of all cases of IE. Risk factors include
IVDA, cardiac abnormalities, and presence of a central venous catheter or prosthetic
valves. Right side of the heart is more commonly involved than the left. 42% of polymicrobial
IE affect the tricuspid valve alone. Emboli are the most common complication presenting
as septic pulmonary focci.
Bacillus
species have been implicated as the most common contaminant in drug paraphernalia,
placing patients with history of IVDA at risk of
Bacillus
infections. When appropriate antibiotic therapy fails to clear bacteremia, surgical
intervention to remove the nidus of infection should be pursued.
POST-CRANIOTOMY RASH AND JAUNDICE
K.M. Coyle
1; K.J. Smith1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115569)
LEARNING OBJECTIVES
1. To recognize the presentation of Anticonvulsant Hypersensitivity Syndrome (AHS).
2. To identify causes, complications and medical management of AHS.
CASE
AS is a 47 year old male with PMH of hypertension who was transferred to our institution
on post-operative day (POD) #32 status-post meningioma resection. He was placed postoperatively
on phenytoin for seizure prophylaxis. On POD #20 AS developed a confluent, whole body,
non-pruritic macular erythematous rash. Phenytoin was discontinued and neurontin prescribed.
Three days later his prescription was changed to phenobarbital. The rash initially
resolved following phenytoin discontinuation but worsened POD #28. On POD #30 he developed
oral ulcerations, generalized edema, yellow sclerae and fever. On presentation to
an outside hospital emergency department he was febrile with painful cervical lymphadenopathy,
generalized macular rash and icteric sclerae. Serum ALT = 1648, AST = 2091, total
bili = 27, NH3 = 53, INR = 1.4 and % eosinophils = 28. He was transferred to our institution
and liver biopsy revealed eosinophilic infiltration consistent with hypersensitivity
reaction. AHS was diagnosed; oral prednisone 40 mg/day was initiated. AS received
supportive intravenous fluid hydration, daily liver function test monitoring and lactulose
to minimize ammonia levels. He was discharged 20 days following admission with normalized
liver function tests, resolved rash, oral lesions and eosinophilia.
DISCUSSION
AHS is an acute, life-threatening, idiosyncratic, non-dose-related drug reaction occurring
1 to 8 weeks after anticonvulsant exposure. The incidence of AHS is 1 in 1,000–10,000
exposures. It is characterized by multisystemic involvement, fever, lymphadenopathy,
mucocutaneous rash, hypertransaminasemia and peripheral eosinophilia. Internal organ
involvement most often involves the liver, but the renal, pulmonary and central nervous
systems may also be affected. Although reported in patients taking lamotigrine, AHS
is associated with the aromatic antileptic drugs phenytoin, carbamazepine, phenobarbital
and primidone. It is thought to be related to inadequate detoxification of arene oxide
metabolites of these drugs by a structural or functional defect in the enzyme epoxide
hydroxylase; the metabolites subsequently cause cell necrosis or a secondary immunologic
response. Cross-reactivity between drugs is 70–80%; there is a familial occurrence
of AHS with an autosomal pattern of inheritance. Management consists of withdrawl
of the offending agent and supportive care. Systemic corticosteroids are successful
in the presence of internal organ involvement. Slow steroid taper is recommended following
resolution of AHS as relapses are reported. Transient hypothyroidism can occur 1–3
months after the initial reaction.
POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME: WHEN A STROKE IS NOT A STROKE
A.B. Benson
1; S. Desai1. 1Oregon Health & Science University, Portland, OR. (Tracking ID #116016)
LEARNING OBJECTIVES
1. Clinically recognize and diagnose posterior reversible encephalopathy syndrome
(PRES). 2. Recognize patients who develop acute renal failure often have rapid elevations
in blood pressure increasing the risk for the development of PRES. 3. Differentiate
PRES from a bilateral posterior cerebral stroke to ensure appropriate acute management.
CASE
A 19 y/o female was admitted to the ICU with fulminant hepatic and acute renal failure
following a suicide attempt with alcohol and acetaminophen. She received N-acetylcysteine
and had rapid improvement in her liver function. From day 2–9 her renal failure, secondary
to acute tubular necrosis, necessitated hemodialysis and caused progressive blood
pressure elevation with episodic mean arterial pressures between 111–117 mmHg. On
day 7, the patient complained of headache, nausea, increasing confusion and blurry
vision rapidly progressing to complete blindness. Her BP was noted to be 150/105.
She then experienced a generalized tonic-clonic seizure and was given lorazepam and
phenytoin. An initial head CT was read as “multiple bilateral cerebellar, occipital
and parietal infarcts.” A subsequent head MRI demonstrated extensive bilateral gray
and white matter edema in the cerebellar, occipital, posterior parietal and temporal
lobes. After neurology consultation, the patient was diagnosed with PRES based on
her initial symptom complex and MRI findings. By discharge, her liver and kidney function
had returned to normal and she remained seizure free without visual or mental deficit.
DISCUSSION
PRES is defined by headache, nausea, vomiting, mental status changes, visual changes
and seizures associated with the characteristic head MRI findings of extensive bilateral
subcortical edema most commonly in the posterior cerebral hemispheres. The two most
common predisposing factors to the development of PRES are acute episodic and sustained
elevations in blood pressure, common in acute renal failure, and/or the use of cyclosporine
and other immunosuppressants. Treatment is to rapidly lower BP to baseline levels,
and if the situation warrants, withdraw or decrease the immunosuppressant dosage and
initiate seizure prophylaxis. This aggressive approach can induce cerebral ischemia
in the setting of an ischemic stroke, but irreversible cases of leukomalacia and progression
to infarction have been described making acute recognition, differentiation and management
of PRES essential. An appropriate clinical scenario coupled with an MRI read by an
experienced neuroradiologist differentiates PRES from a stroke and potentiates appropriate
treatment. Symptoms usually resolve without sequelae in days to weeks.
POSTPARTUM CARDIOMYOPATHY
H. Shishodia1; J. Miller1; M. Bohning
1. 1Temple University, Philadelphia, PA. (Tracking ID #116444)
LEARNING OBJECTIVES
1. Recognize the clinical manifestation of congestive heart failure (CHF). 2. Recognize
postpartum cardiomyopathy as an infrequent but known cause of CHF that can present
up to 6 months after delivery. 3. Discuss the implications of medical management of
postpartum cardiomyopathy.
CASE
A 22 year old G1P1 woman presented to outpatient clinic 6 weeks postpartum with progressive
dyspnea on exertion, and now short of breath at rest. She reported a non-productive
cough, orthopnea, paroxysmal nocturnal dyspnea and lower extremity edema. Her medical
history is significant only for a pregnancy complicated by preeclampsia and peripartum
hemorrhage requiring transfusion. On exam her was BP 112/90 P 118, RR 24, Pulse Ox
99% on room air. She had gained 5 lbs. since her last clinic visit a month prior.
She was able to speak in full sentences at rest. Her lungs were clear, she had 11
cm JVP. She had a laterally displaced hyperdynamic PMI, right ventricular heave, III/VI
blowing systolic murmur radiating to the axilla, an S3 and bilateral 2+ pitting edema
above the ankles. EKG showed sinus tachycardia. The patient was hospitalized and a
bedside echocardiograph showed 4 chamber dilatation, depressed left ventricular systolic
function of 10–15%, severe mitral and tricuspid regurgitation, mild aortic regurgitation
and moderate pulmonic regurgitation. Hgb 11.5, Hct 35.5, TSH 4.49. She was given diuretics,
an ACE-inhibitor and digoxin and her symptoms improved dramatically.
DISCUSSION
Postpartum cardiomyopathy is a dilated cardiomyopathy which results in signs and symptoms
of heart failure. It is an uncommon cardiomyopathy with an incidence of 1/3000–1/4000
births. Symptoms typically begin during the last trimester of gestation and the diagnosis
is usually made in the early peripartum period, but can develop upto 6 months after
delivery. Common signs and symptoms include shortness of breath, fatigue, chest pain,
palpitations, peripheral edema. Physical exam usually demonstrates an enlarged heart,
S3, murmurs of mitral and tricuspid regurgitation. Echocardiography usually shows
enlargement of all four chambers, with severe reduction in left ventricular systolic
function. Multiparous women, women with preeclampsia and twin pregnancies are at a
higher risk for peripartum cardiomyopathy. Acute management involves oxygen, diuretics,
digitalis and vasodilators. Other modalities such as dopamine, dobutamine, milrinone
have been used in pregnant women in a few cases. There is a high risk of mortality
in patients with severe heart failure who do not recover and these women are referred
for cardiac transplant. Risk of mortality ranges from 0–2% when the left ventricular
function has normalized, while the risk of mortality is 8–17% in women with depressed
systolic function prior to subsequent pregnancy. It is for these reasons that subsequent
pregnancies are deemed high risk and therefore discouraged.
POST-PARTUM MASTITIS AND CONSEQUENCE OF DELAYED INTERVENTION
K. Ghosh
1; A.C. Degnim1; K.R. Brandt1. 1Mayo Clinic, Rochester, MN. (Tracking ID #116645)
LEARNING OBJECTIVES
1) Emphasize the significance of imaging tests in the assessment of postpartum mastitis.
2) Address the role of antibiotics in the treatment of postpartum mastitis. 3) Emphasize
supportive care for women with evidence of postpartum mastitis.
CASE
A 24 year-old primiparous woman presented with recurrent postpartum mastitis, and
persistent pain and redness of the breast. The patient first noticed a lump in the
inner left breast about nine weeks after childbirth that was associated with redness
and warmth. Symptoms improved spontaneously but recurred soon after and she was evaluated
by her primary physician and advised to start Cephalexin with some improvement. After
three weeks on Cephalexin, she noticed drainage from the midst of the area of erythema,
and was switched to Amoxicillin/Clavulanate and sent to the Breast Clinic for further
treatment. Examination revealed an asymmetrically enlarged, tender, engorged, left
breast with a 5 cm area of redness and warmth in the infero-medial quadrant. Ultrasound
evaluation revealed a fluid collection measuring over 10 cm in the left breast with
features suggestive of an infected galactocele/breast abscess. She underwent incision
and drainage of the abscess with removal of approximately 160 cc of pus, and debridement
of the abscess wall. The cavity measured 15 × 10 cm. Post-operatively, the abscess
cavity was packed frequently enabling complete wound healing in four weeks. The patient
is now contemplating plastic surgery to improve cosmesis.
DISCUSSION
Postpartum mastitis can present a clinical spectrum from mild focal breast inflammation
to breast abscess formation and sepsis, occurring in 2 to 33% of breast-feeding women.
Management of postpartum mastitis includes supportive measures such as hot compresses,
analgesics and the expression of breast milk either by breast feeding, manual expression
or use of a breast pump. In mild mastitis, these measures often suffice to resolve
symptoms. However, breast abscess and septicemia are known complications of post-partum
mastitis, and therefore, the role of antibiotic therapy has been an area of controversy.
Ultrasound evaluation of the area of mastitis is recommended to rule out an underlying
abscess, or infected galactocele. The presence of an infected fluid collection requires
treatment with drainage as well as antibiotic therapy to prevent the spread of infection
and enlargement of the abscess cavity.
PRESCRIBING SELECTIVE SEROTONIN REUPTAKE INHIBITORS IN THE ELDERLY: WATCH OUT FOR
MENTAL STATUS CHANGES
A.L. Puswella
1; K. Barnard1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116219)
LEARNING OBJECTIVES
1) To recognize the Syndrome of Inappropriate ADH Secretion (SIADH) as a common side
effect of selective serotonin reuptake inhibitor (SSRI) therapy. 2) To recognize the
importance of early detection of SIADH in elderly patients starting SSRI therapy.
3) To recognize the risk factors that predispose patients to developing SSRI-induced
SIADH.
CASE
An 88 y/o female was brought to the emergency department by her neighbor for increasing
confusion, weakness, and altered mental status for two days. Past medical history
is significant for depression, hypertension, hyperlipidemia, osteoporosis and urinary
incontinence. Her medications included Losartan, Propanolol, Alendronate, Tolterodine.
She had been started on Escitalopram 10mg two days prior to the onset of symptoms.
In the emergency department, her serum sodium was 118 mEq/L and she was judged to
be euvolemic. Her baseline serum sodium, taken two days earlier was 134 mE/L. Urine
electrolytes showed Na+= 96 mEq/L, Cl = 114 mEq/L and osmolarity = 447 mOsmol/kg.
A diagnosis of SSRI-induced hyponatremia due to SIADH was made. The patient was admitted,
placed on fluid restriction, and the Escitalopram discontinued. Her mental status
gradually improved, returning to baseline over the next five days.
DISCUSSION
The development of SIADH secondary to SSRI use in the elderly appears to be a more
common occurence than previously thought. Some series show an incidence as high as
28%, with severe symptomatic hyponatremia occuring in 12% of these cases. Most episodes
occur within 13 days (range 3–120 days) from the onset of treatment. Our patient demonstrates
that severe symptomatic hyponatremia can occur in as little as two days, and can result
in expensive hospitalization. Elderly women on multiple medications are at especially
high risk of developing SIADH. The reasons for this are the higher rates of depression
and SSRI use, decreased kidney function, and polypharmacy, especially the concomitant
use of thiazide diuretics and neuroleptics. This case demonstrates the need for close
monitoring of mental status and serum sodium when initiating SSRI drugs in the elderly.
Females and those taking thiazide diuretics or neuroleptic medications appear to be
at higher risk.
PRIMARY CHORIOCARCINOMA OF THE STOMACH WITH LIVER METASTASES—A CASE REPORT
A. Devarajan1; K. Subramanian
1; C. Rathnakumar1; A. Regaila1; E. Christina2; U. Hitendra1; R. Jeyachandran1. 1Jersey
City Medical Center, Jersey City, NJ; 2JerseyCity Medical Center, Jersey City, NJ.
(Tracking ID #117440)
LEARNING OBJECTIVES
Choriocarcinoma is a malignant proliferation of the Langerhans cells and of syncytial
cells of trophoblastic origin that is normally situated in the female genital tract
after a gestational event such as molar pregnancy, term pregnancy, abortion or ectopic
pregnancy. Rarely it occurs in either sex as a midline lesion in the retroperitoneum,
mediastinum and the region of the pineal gland. Less frequently it is found in the
bladder, liver, stomach and colon, where it is seen in combination with adenocarcinoma.
Primary choriocarcinoma of the stomach is an extremely rare and highly malignant tumor.
CASE
We report a case of choriocarcinoma of the stomach associated with adenocarcinoma
in a 51 year old female, who presented with epigastric pain and vomiting for one month
with manifestations of liver failure and elevated Ò-HCG (38748 MIU/ml). No evidence
of gestational malignancy was found on endometrial biopsy. Upper GI endoscopy revealed
a single acute friable ulcer in the antrum. CT scan of the abdomen showed multiple
liver metasteses. Histopathologic examination of gastric antral and liver biopsy specimens
demonstrated ulcerated, infiltrating choriocarcinoma with poorly differentiated adenocarcinoma.
The diagnosis was further confirmed by immunohistochemical staining, which showed
strong positivity for Ò-HCG, CEA and CAM 5.2, and focal positivity for AFP, CK and
EMA. The patient was treated with one cycle of methotrexate based chemotherapy, as
patient refused dactinomycin and VP-16 based chemotherapy. The level of serum Ò-HCG
was reduced (7311 MIU/ml) with no parallel clinical improvement. Patient continued
to deteriorate and expired within one month of diagnosis.
DISCUSSION
Primary choriocarcinoma of the stomach presents with a picture similar to adenocarcinoma
with an average age of onset of 50 to 60 years with greater preponderance among males.
It spreads by hematogenous route with an average survival of only a few months from
the time of diagnosis. Although chemotherapy is successful in treating gestational
choriocarcinoma, its effectiveness had not been shown in gastric choriocarcinoma.
Standardized treatment protocols are lacking due largely to the paucity of cases presented
at any one institute. This calls for an organized multicenter or multinational double
blind, case-control randomized study in order to establish a treatment protocol for
this life threatening malignancy.
PULMONARY CRYPTOSPORIDIOSIS
L. Subramanyam
1; S. Parikh1; M. Eapen1; H. Friedman1. 1St. Francis Hospital, Evanston, IL. (Tracking
ID #116549)
LEARNING OBJECTIVES
1. Recognize the importance of Cryptosporidium species not only as a cause of intractable
diarrhea and malabsorption in immunocompromised patients but also as an agent that
leads to respiratory failure and death. 2. Realize that early immune reconstitution
with Highly Active Anti-Retroviral Therapy (HAART) and better nutrition improves the
final outcome in patients with cryptosporidiosis and Acquired Immune Deficiency Syndrome
(AIDS); prognosis without immune restoration is generally poor.
CASE
The patient is a 34-year-old African-American man who presented with a 4 week history
of progressively worsening shortness of breath, cough associated with mucoid sputum,
moderate epigastric pain, loose watery diarrhea and a weight loss of 30 lbs during
this period. He appeared emaciated with obvious clinical signs of dehydration and
moderate respiratory distress, saturating 88% on room air. Cardiac and lung exam was
essentially benign. The abdomen was diffusely tender to palpation without any evidence
of peritoneal signs. He tested positive for HIV with a viral load of 685,354 and a
CD4 count of 177. Chest X-ray showed diffuse interstitial infiltrates. He was started
on HAART and Trimethoprim-sulfamethoxazole empirically for Pneumocystis carinii pneumonia.
Stool specimens and a subsequent duodenal biopsy demonstrated Cryptosporidial oocysts.
During the course of hospital stay, his respiratory symptoms worsened. Pneumocystis
was persistently negative in the induced sputum, whereas the sputum sent for AFB staining
revealed Cryptosporidia in three consecutive specimens. He was treated with paramomycin,
azithromycin and intravenous hyperalimentation. Despite aggressive treatment, he succumbed
to the infection and died of respiratory failure.
DISCUSSION
While there is a well-documented association of Cryptosporidium with severe diarrheal
disease in immunosuppressed individuals, Cryptosporidium in the respiratory tract
has been rarely described. The few case reports that are available in the literature
show that these patients ultimately develop fulminant respiratory failure and die.
But, the causal association could not be clearly established in these cases because
of the presence of other respiratory pathogens especially, Pneumocystis. In our patient,
we could not isolate any other microorganism even with multiple sputum studies. So,
we consider this case as one of the rare reported cases of pulmonary cryptosporidiosis
with an adverse outcome. The risk of fulminant cryptosporidiosis increases in profoundly
immunocompromised patients with AIDS as measured by the low CD4 count. Immune reconstitution
with HAART has shown to decrease mortality and morbidity in these individuals. Early
consideration of this diagnosis and prompt institution of treatment might improve
the final outcome in these patients.
PULMONARY HYPERTENSION: IS IT FIRST, OR IS IT SECOND?
A. Toprani
1; J. Hutchings1. 1Tulane University, New Orleans, LA. (Tracking ID #117519)
LEARNING OBJECTIVES
1. Recognize the clinical presentation of primary pulmonary hypertension. 2. Understand
the criteria for diagnosis and treatment of primary pulmonary hypertension.
CASE
A 31 year-old woman presented with hemoptysis. She had no history of trauma or anticoagulation,
and no recent tuberculosis exposures. She had presented to an outpatient clinic six
months earlier with similar complaints and was prescribed an antibiotic. Her symptoms
resolved, but the hemoptysis recurred one week prior to presentation. She had a III/VI
diastolic murmur; the remaining exam including a PPD test was normal. The chest radiograph
revealed cardiomegaly and clear lungs. An echocardiogram revealed a pulmonary artery
pressure of 130 mmHg. Serologies for HIV, RF and ANA were negative. PFT's were normal.
Bronchoscopy was negative, and a HRCT was negative for parenchymal lung disease. The
V/Q scan was normal. A right heart catheterization showed a positive response to epoprostenol.
DISCUSSION
Primary pulmonary hypertension (PPH) is a pulmonary artery pressure of greater than
25 mmHg in the absence of an identifiable cause. The pathogenesis is related to an
imbalance between vasodilating and vasoconstricting factors, leading to pulmonary
vasoconstriction and vascular remodeling. A mutation of the bone morphogenetic protein
receptor type II (BMPR2) gene has been identified in the familial form of PPH. Many
of the sporadic cases of PPH are also attributable to abnormalities of BMPR2. In order
to diagnose primary pulmonary hypertension secondary causes must be excluded. This
is accomplished by performing pulmonary function tests, connective tissue serologies,
echocardiography, cardiac catheterization and ventilation/perfusion lung scanning.
Cocaine, amphetamines and the appetite suppressant fenfluramine should also be excluded.
The response to epoprostenol or nitric oxide should be investigated. The 30% of patients
who do respond can be treated with oral calcium channel blockers. Patients who do
not qualify for oral therapy are treated with continuous intravenous infusion of epoprostenol.
Chronic anticoagulation with warfarin has been shown to improve survival.
PURULENT PERICARDITIS: A RARE COMPLICATION OF STREPTOCOCCAL PNEUMONIA
H. Bhuria1; M. Karmegam
1; H. Friedman1. 1Saint Francis Hospital of Evanston, Evanston, IL. (Tracking ID #116654)
LEARNING OBJECTIVES
Recognize purulent pericarditis as a complication of pneumococcal pneumonia.
CASE
A 55 year old white female with history of alcohol abuse presented with nausea, vomiting
and RUQ pain for 4 days, followed by dry cough and right sided pleuritic chest pain.
She denied fever or shortness of breath. On exam temperature 97 F, pulse 122, BP 135/115,
RR 28, no JVD, S1S2 audible and decreased breath sounds at bases. Abdomen showed mild
RUQ tenderness but no Murphy's sign. CXR showed bibasilar effusions and infiltrates
with cardiomegaly. CT abdomen was unremarkable. Blood culture subsequently grew Streptococcus
pneumoniae. She was started on IV antibiotics for pneumonia. The next day she developed
shortness of breath and hypotension. A chest CT scan showed bilateral pleural and
pericardial fluid. Cardiac echocardiogram showed tamponade. 450 cc of purulent fluid
was drained from the pericardial cavity. Analysis revealed white cell count of 56,700
with 92% neutrophils and a negative bacterial culture. Both pleural cavities were
drained by tube thoracostomies aided by repeated instillation of tPA. Plerual fluid
showed a white cell count of 2,600 with 89% neutrophils, ph 6.8, glucose 2, LDH 12,540
and a negative bacterial culture. She required a pericardial window for reaccumulating
fluid and was eventually discharged in a stable condition.
CT chest showing bilateral pleural effusions (R > L) and pericardial effusion.
DISCUSSION
Purulent pericarditis in the antibiotic era has become a rare entity. Prior to the
widespread use of antibiotics, purulent pericarditis was usually a complication of
pneumococcal pneumonia; in modern times, most cases are associated with dialysis,
thoracic surgery, and chemotherapy. It is occasionally seen in patients with risk
factors for invasive pneumococcal disease like alcoholism. A high index of suspicion
should be maintained in patients with pneumonia and pericardial effusion as prompt
diagnosis and treatment with drainage of pericardial cavity is essential for a good
outcome.
PYODERMA GANGRENOSUM OF THE HANDS FOLLOWING VENIPUNCTURE INJURY
C. Nassaralla
1; R. McCurdy1; S. Frost1. 1Cleveland Clinic Foundation, Cleveland, OH. (Tracking
ID #115494)
LEARNING OBJECTIVES
1) Diagnose and treat pyoderma gangrenosum. 2) Recognize that pyoderma gangrenosum
is often misdiagnosed as bacterial skin infection, potentially resulting in devastating
outcomes due to delay in therapy.
CASE
A 28-year-old woman was admitted to the hospital with excruciatingly painful hand
ulcers. Eighteen days prior to presentation, she had experienced multiple traumatic
attempts at intravenous (IV) access on the dorsum of both hands before undergoing
cholecystectomy. After surgery, small blisters appeared at the attempted IV access
sites that rapidly enlarged and ulcerated. Five days prior to admission she received
amoxicillin-clavulanate for the presumptive diagnosis of impetigo, yet the ulcers
continued to enlarge. Physical examination revealed tender 10 cm and 6 cm circular
ulcers with hemorrhagic exudate on the dorsal aspects of the left and right hands
respectively. The ulcers were demarcated with an irregular, raised, and boggy border.
The surrounding skin was erythematous and edematous. The ulcers did not appear infected,
and blood and wound cultures were sterile. There was no fever, and laboratory evaluation
was remarkable only for mild leukocytosis. Prednisone and pain medications were prescribed
after pyoderma gangrenosum (PG) was diagnosed based on clinical history, and typical
ulcer appearance. Extensive evaluation revealed no associated systemic illness, and
the ulcers were well healed after 14 days of therapy.
DISCUSSION
PG is a rare, idiopathic inflammatory neutrophilic dermatosis akin to Sweet's syndrome.
Most cases are associated with a systemic illness such as inflammatory bowel disease.
However, 15% to 30% of patients have no underlying medical condition. The pathogenesis
of PG is unclear, but altered immunological reactivity is likely operative. PG lesions
begin as tender, red macules, papules, nodules, or bullae that evolve into pustules
or vesicles surrounded by erythematous and edematous skin. Ulceration eventually occurs,
which then extends in a centrifugal pattern. PG confined to the hands is rare and
has been variably referred to as “neutrophilic dermatosis of the dorsal hands”, “pustular
vasculitis of the hands”, and “variant erythema elevatum diutinum.” Diagnosis of PG
is based on clinical findings, including a history of minor trauma as the precipitating
event, which is commonly associated with PG of the hand. Biopsy specimens generally
yield nonspecific results. Systemic immunosuppressive therapy with prednisone is the
cornerstone of therapy. Dapsone may be useful in decreasing inflammation and edema.
Surgical procedures are contraindicated, as they may provoke a pathergic response
that exacerbates tissue injury. PG is frequently misdiagnosed as bacterial skin infection,
and antimicrobial therapy is ineffective. Inaccurate diagnosis and delay in appropriate
treatment can result in devastating outcomes such as amputation of the affected areas.
RASH, ABDOMINAL PAIN AND WEIGHT LOSS
S. Prall
1; G. Babameto2. 1Geisinger Medical Center, Danville, PA; 2Geisinger Mediacal Center,
Danville, PA. (Tracking ID #115169)
LEARNING OBJECTIVES
1. Recognize atypical presentation of adult onset diabetes. 2. Diagnose hypertriglyceridemia
related to uncontrolled diabetes.
CASE
A 41 yo white male presented with complaints of abdominal pain. Over the past few
months he had lost 40 pounds. One month ago he developed a nontender rash on his extremities
and trunk. 24 hours ago he developed pain after eating supper, not relieved with over
the counter analgesics. Denied nausea, vomiting, diarrhea, or fevers. PMH included
reflux, hiatal hernia, carpal tunnel syndrome b/l. No tobacco or alcohol use. Currently
disabled from carpal tunnel syndrome. Physical exam: BP 130/73, HR 87, RR 20, T 37.5.
Obese. HEENT: sclera anicteric. Remarkable for tense, distended abdomen with epigatric
tenderness, normal bowel sounds, no rebound. Yellow nontender papules varying in size
on extremities and trunk (xanthomata). Initial laboratories: lipase 1,468, cholesterol
401, triglyceride 14,798, ast 31, alt 59, alkaline phosphatase 80, total bilirubin
0.5, WBC 12.8, Na 125, K 3.6, glucose 325, CO2 21, TSH 2.04. Pt was admitted for management
of acute pancreatitis likely secondary to his hypertriglyceridemia resulting from
uncontrolled diabetes. Initial HgA1C was 14.2. With insulin therapy HgA1C improved
to 5.8, however, cholesterol, LDL, and TG remained elevated requiring lipid lowering
medications.
DISCUSSION
Pancreatitis is an uncommon presentation for new adult onset diabetes. Frequently
patient's present with hyperglycemia, obesity, ketonuria, weight loss, nonketotic
acidosis. Hyperlipidemia is frequently seen in uncontrolled diabetes, specifically
hypertriglyceridemia. Hyperinsulinemia plus insulin resistance in uncontrolled diabetes
cause increased TG production coupled with reduced clearance of plasma TG result in
hypertriglyceridemia. Hypertriglyceridemia is a rare cause of pancreatitis. Levels
greater then 1,000 place an individual at increased risk for pancreatitis. The hypertriglyceridemia
resulting from pancreatitis is mild to moderate and should not be confused with the
markedly elevated levels seen causing pancreatitis. Significantly elevated TG levels
in the uncontrolled diabetic should also raise the question of an underlying lipid
disorder. In this case the patient presented with hypertrigylceridemia induced pancreatitis
and was found to have diabetes, but we can not assume that the diabetes alone resulted
in his clinical presentation. The presence of xanthomas indicates that he likely had
a longstanding hyperlipidemia. The concomitant development of diabetes probably exacerbated
the preexisting hyperlipidemia. Despite ideal glycemic control with a follow up HgA1C
of 5.8, the patient's lipid profile failed to normalize and he continued to require
lipid lowering agents. Think of hypertriglyceridemia induced pancreatitis in the uncontrolled
diabetic who presents with abdominal pain.
RECOGNIZING MAC TO AVOID THE KNIFE
S. Kahlon
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117476)
LEARNING OBJECTIVES
1. Recognize a complication of a common AIDS-related opportunistic infection, disseminated
mycobacterium avium-intracellulare complex (MAC).
CASE
A 32 year-old HIV-positive man (CD4 = 28) presented with four days of progressively
worsening diffuse, crampy abdominal pain with fever, night sweats, vomiting, and inability
to tolerate oral intake. His examination was normal with the exception of a temperature
of 38.5°C. He had mild abdominal tenderness without guarding. An initial radiograph
of the abdomen suggested small bowel obstruction versus adynamic ileus. As his symptoms
did not resolve, he was scheduled for exploratory laparotomy. In preparation, a CT
scan of the abdomen was performed that revealed diffuse retroperitoneal and mesenteric
lymphadenopathy with a partial ileus. A subsequent CT-guided needle biopsy of a retroperitoneal
node revealed reactive lymphadenopathy due to Mycobacterium avium. Supportive care
for nausea and pain was provided, as well bowel rest and nutritional support, until
he was again able to tolerate oral intake, began having bowel movements, and symptomatically
improved.
DISCUSSION
Mycobacterium avium (MAC) bacteremia can occur in HIV-positive patients with CD4 counts
less than 50. The syndrome includes fever, weight loss, night sweats, diarrhea, and
lymphadenopathy. Organs involved include the liver, spleen, gastrointestinal tract,
lymph nodes, and bone marrow. Pancytopenia, elevated lactate dehydrogenase, and elevated
alkaline phosphatase are laboratory indicators for the disease. The organism has a
predilection for lymph nodes and can induce a diffuse reactive lymphadenopathy that
can be sufficient to cause obstruction of contiguous organs. Gastrointestinal lymphadenopathy
can be symptomatic due to mass effect and inflammation of the bowel wall. Physicians
should be aware of this common complication from disseminated MAC, as unlike its imitator,
lymphoma, it is readily contained with ethambutol and clairthromycin.
RECURRENT ABDOMINAL PAIN: CHECK THE MEDICATION LIST
J. Kamali
1; G.L. Arnold1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116496)
LEARNING OBJECTIVES
1) To recognize angiotensin-converting enzyme (ACE) inhibitors as a cause of angioedema
of the small bowel. 2) To list the typical symptoms of angioedema of the small bowel.
CASE
A 49 year-old African American woman with past medical history of essential hypertension
and recurrent abdominal pain for two years presented to the emergency department (ED)
with progressive abdominal pain of four hours duration. The pain was periumbilical,
constant, sharp and without radiation. It started at home as 2/10 severity and progressed
to 6/10 in the ED. She had two similar episodes within the past two years, one requiring
exploratory laparoscopy. The patient reported associated nausea, but denied vomiting,
diarrhea, constipation, bloody stools, fever or chills. On further questioning she
recalled previous episodes of swelling of her lips lasting several days, beginning
after two months of intermittent abdominal symptoms. Her medications included Lotrel®
(amlodipine + benazepril), iron sulfate and occasionally alprazolam. Physical examination
was notable for normal vital signs. Her sclera were anicteric. Heart and lung examination
was unremarkable. Abdomen was soft and non-distended. There was moderate periumbilical
tenderness without guarding. Her laboratory studies showed WBC of 15.5, lipase 61
and amylase 89. CT of the abdomen demonstrated edematous loops of the small bowel.
C1 esterase inhibitor level as well as C1 and C2 levels were normal.
DISCUSSION
Angioedema has been reported to occur in 0.2% of patients taking ACE inhibitors. It
usually involves the deep layers of the skin but may also occur at the mucosal surfaces
of the upper respiratory tract or gastrointestinal (GI) tract. The clinical presentation
of the angioedema involving the GI tract includes abdominal pain, nausea, vomiting,
diarrhea and/or ascites. There have been nine previous case reports of ACE inhibitor
induced angioedema of the small bowel. CT of the abdomen demonstrating small bowel
edema can suggest the diagnosis. The time between starting the medication and the
onset of GI tract symptoms has been reported to range from a few hours to four months,
but in most cases is less than seven days. The patient in this case was started on
benazepril two months prior to the first episode of the abdominal pain. Failure to
think of this diagnosis can result in delay of diagnosis and unnecessary procedures,
such as exploratory laparoscopy and even bowel resection. No prior case reports have
included angiotensin inhibitor level measurements during the acute episode. Angioedema
of the GI tract caused by ACE inhibitors is probably underdiagnosed and underreported.
It should be considered in all patients taking ACE inhibitors who present with the
typical abdominal symptoms.
RECURRENT EPISODIC CHEST PAIN IN A WOMAN WITH INFERTILITY
L.N. Dyrbye
1; C. Rohren1. 1Mayo Clinic, Rochester, MN. (Tracking ID #101799)
LEARNING OBJECTIVES
Learning objectives 1. Clinical recognition of catamenial hemothorax 2. Treatment
of catamenial hemothorax
CASE
40 year old woman presented with two days of dull, non-pleuritic right chest pain
aggravated by recumbency on the right side, with associated dyspnea. She reported
monthly chest pain since 1993. No fever, chills, or cough. Her period started five
days ago. The patient's past medical history included infertility and right-sided
hydropneumothorax in 2001 with biopsy proven pleural and peritoneal endometriosis
and diaphragmatic fenestration on thoracotomy. Diaphragmatic repair and chemical pleurodesis
were performed. On exam, her vital signs were normal. Lung sounds were diminished
over the right upper lung. Chest x-ray showed a small, right-sided hydropneumothorax.
After consultation with thoracic surgery, the patient elected to undergo hormonal
treatment.
DISCUSSION
Catamenial thoracic syndromes (CTS) are rare. Clinically, women between 30–34 years
of age present with chest pain and dyspnea that starts within the first two days of
menstrual flow. Hemoptysis is rarely reported. Chest radiograph commonly shows right-sided
pneumothorax, occasionally hemothorax, and rarely nodules. [1][2] Long-term therapy
involves suppression or removal of existing endometrial plaques and prevention of
further plaque development. Hormonal suppression with oral contraceptives, progesterone,
GnRH, and danazol is unsuccessful long-term. CTS recur in up to 50%. [1] Surgical
treatment includes removal of endometrial plaques and mechanical or chemical pleural
abrasion. Resection of endometrial implants on the pleura and diaphragm, along with
repair of diaphragmatic abnormalities have had variable short-term success. [2][3]
Chemical pleurodesis is more successful than hormones in preventing recurrence. [1]
Regardless of the surgical method used, cyclical chest pain, however, may remain.
[1] Surgery followed by GnRH to prevent recurrence and pain maybe the best option.
[4] 1. Joseph, J.M.D. and S.A.M.D. Sahn, Am J Med 1996. 100(2): p. 164–170. 2. Alifano,
M., et al., Chest, 2003. 124(3): p. 1004–8. 3. Sakamoto, K., T. Ohmori, and H. Takei,
Ann Thorac Surg, 2003. 76(1): p. 290–1. 4. Blanco, S., et al., J Thorac Cardiovasc
Surg, 1998. 116(1): p. 179–80.
RECURRENT PARAPNEUMONIC PLEURAL EFFUSIONS AND WORSENING DYSPNEA IN A YOUNG WOMEN
N. Latif
1; G.H. Tabas1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115832)
LEARNING OBJECTIVES
Consider non-pneumonic causes of dyspnea and pleural effusion in a patient with a
history of pneumonia.
CASE
A 34-year-old Caucasian woman with a history asthma, reflex sympathetic dystrophy
and obesity was referred from another institution because of recurrent bilateral pleural
effusions one year after an episode of pneumonia. Her symptoms included worsening
dyspnea, pleuritic chest pain and a 100-pound weight loss. Physical examination showed
bilaterally decreased breath sounds and ankle edema. Her chest x-ray revealed bilateral
pleural effusions and computerized tomography of the chest revealed a small pericardial
effusion and no pulmonary embolism. Examination of the pleural fluid showed it was
transudative and pulmonary function testing demonstrated restrictive lung disease.
An echocardiogram showed a normal ejection fraction and a dilated inferior vena cava.
Abdominal ultrasound revealed dilated intrahepatic ducts and hepatic congestion. Right
heart catheterization showed equalization of the right ventricular diastolic pressure
and the pulmonary diastolic pressure. The pulmonary capillary wedge pressure was 20
mmHg. The patient had symptomatic improvement after pericardiectomy and pathologic
examination revealed fibrosis and hyalinization.
DISCUSSION
Post-pneumonic pericarditis is a rare complication of pneumonia but important to diagnose.
The major categories of constrictive pericarditis are idiopathic, post-radiotherapy
and post-cardiac surgery. Infectious causes account for only 6% of cases of constrictive
pericarditis. Patients with constrictive pericarditis usually present with congestive
heart failure but rarely with cardiac tamponade. Classic clinical sign are increased
jugular venous pressure, peripheral edema, pulsatile liver, pulsus paradoxus, Kussmauls
sign and pericardial knock. Diagnostic testing includes electrocardiogram showing
low voltage and echocardiography showing pericardial effusion and pericardial thickening.
Computerized tomography of the chest shows pericardial thickening. Right heart catheterization
reveals equalization of right-sided pressures. The treatment is pericardiectomy.
RECURRENT SYNCOPE AS A RARE SYMPTOM OF MASTOCYTOSIS
A.T. Czajka-Giermasz
1; W.N. Kapoor1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116571)
LEARNING OBJECTIVES
1) to recognize systemic mastocytosis (SM) as a rare cause of syncope 2) to manage
patients with SM.
CASE
A 44-year old white female was transferred to the MICU after her fourth episode of
syncope. Three weeks prior, she experienced sudden onset of flushing followed by severe
lightheadedness and palpitations. Her husband found her unresponsive and incontinent
of feces. When paramedics arrived 20 minutes later she was hypotensive, tachycardiac,
confused, and could not follow commands. Initial work-up revealed normal EKG, head
CT, chest CT, echocardiogram, CBC and electrolytes. One week later, while eating dinner,
she felt flushed and dizzy, and slumped to the floor, losing consciousness. Cardiac
catheterization, tilt-table test, electrophysiologic studies, thyroid studies, urine
metanephrines, 5-HIAA, and serum serotonin levels were normal. She continued to intermittently
experience flushing and headache. Her third episode of syncope, preceded by flushing,
nausea, and vomiting, occurred a week later. After admission she had another severe
episode of prolonged and profound hypotension with syncope, requiring transfer to
the MICU. At that time the history of urticaria pigmentosa in her daughter was obtained,
and the patient was found to have dermatographism on physical examination. Abdominal
CT scan, brain MRI, VIP, and cortisol were normal. She had elevated serum histamine,
tryptase, and urine histamine levels. Bone marrow biopsy revealed increase in mast
cell numbers, with aggregates of spindle-shaped mast cells, consistent with SM. Treatment
with cromolyn, H1-blocker, and H2-blocker was started. She continued to experience
symptoms, and required massive doses of montelukast, H1 and H2-blockers, and cromolyn
for adequate symptomatic control.
DISCUSSION
Mastocytosis is a disorder of mast cell proliferation. Intense vascular collapse is
caused by spontaneous, or induced mast cell degranulation, and release of vasoactive
madiators like histamine, prostaglandin D2, and leukotriens. Loss of consciousness
is usually accompanied by prodromal symptoms of flushing, palpitations, and lightheadedness.
Patients also can experience pruritus, gastrointestinal and neurologic symptoms. In
the absence of characteristic skin lesions, diagnosis is difficult, but should be
suspected in the patient with unexplained hypotensive episodes and flushing. The evaluation
of the patient with suspected SM includes skin inspection, measurement of markers
of mast cell activation, and bone marrow biopsy. There is no current cure for SM.
Successful treatment of symptoms may require massive doses of H1 and H2-blockers,
steroids, montelukast, and cromolyn.
RENAL ARTERY STENOSIS IN A PATIENT WITH UNCONTROLLED HYPERTENSION
T.T. Tran
1; L.B. Lu2. 1Baylor College of Medicine, Houston, TX; 2Baylor College of Medicine,
Friendswood, TX. (Tracking ID #116996)
LEARNING OBJECTIVES
1) Recognize the importance of workup for secondary causes of hypertension. 2) Review
the presentation, diagnosis, and treatment options of renal artery stenosis.
CASE
A 50-year-old man with a ten month history of hypertension and chronic renal insufficiency
presented with sudden onset of severe shortness of breath and diffuse diaphoresis.
He had been admitted 6 times for similar symptoms due to hypertensive urgencies despite
being on multiple antihypertensive agents. On this admission, his vital signs revealed
BP 248/108, HR 85, RR 16, and temperature 98.1 F. Physical examination was unremarkable.
Specifically, there were no papilledema, abdominal bruits, or peripheral edema. Blood
work was significant for Cr of 1.9, which was at his baseline. Due to his history
of multiple admissions with hypertensive urgencies, the patient underwent a workup
for secondary causes of hypertension that showed no evidence of hyperaldosteronism,
thyroid disease, pheochromocytoma, or Cushing's syndrome. A renal ultrasound revealed
a small left kidney of 8 cm and a normal-sized right kidney of 11 cm. Subsequent renal
arteriogram showed total occlusion of the left renal artery and 90% stenosis of the
right renal artery. After undergoing an angioplasty with stent placement in the right
renal artery, his blood pressure came under better control.
DISCUSSION
Secondary hypertension accounts for 5–10% of hypertensive cases. Features suggesting
of secondary hypertension include age at onset <30 or >55, abrupt onset, > stage 3
(160/100) hypertension, and resistance to effective medical therapy. Causes include
renal insufficiency, renal artery stenosis, coarctation of the aorta, aldosteronism,
Cushing's syndrome, pheochromocytoma, and thyroid disease. Renal artery stenosis accounts
for 0.2 to 4% of all hypertension cases and can be due to atherosclerosis (70%) or
fibromuscular dysplasia (30%). Renal arteriography is the diagnostic gold standard.
The captopril test has a sensitivity of 79% and specificity of 89%. Renography after
captopril has sensitivity and specificity of about 85%. Duplex ultrasonography achieves
sensitivity and specificity of about 90%. Both spiral computed tomographic angiography
and gadolinium-enhanced three-dimensional magnetic resonance angiography are excellent
noninvasive diagnostic tests with sensitivity and specificity of about 95%. There
is not a specific recommended screening non-invasive test for renal artery stenosis;
the clinical index of suspicion should determine the degree of evaluation. Angioplasty
or surgery has been shown to modestly improve blood pressure, but not renal functions
in the presence of renal insufficiency. In summary, refractory hypertension should
warrant the work up for secondary causes.
RENAL TUBERCULOSIS
C. Patel1; M. Taswin
1; V. Bengualid1. 1St. Barnabas Hospital, Bronx, NY. (Tracking ID #117555)
LEARNING OBJECTIVES
In USA and New York State, Genitourinary Tuberculosis is the fourth most common form
of extrapulmonary tuberculosis. It represents 6% of all EPTB.
It is easily overlooked as it usually presents with symptoms of a bacterial pyelonephritis.
Key presentation includes recurrent urinary tract infections despite treatment or
sterile pyuria. Imaging studies (plain film, CT scan, IVP, or USG) can show calcification,
ureteral dilation or obstruction. Diagnosis made by: AFB stain/culture from urine
or biopsy (sensitivity 43,3% and specificity 100%) or PCR of MTB DNA in urine (sensitivity
53.8% and specificity 96.5%).
Renal involvement includes: tubulointerstitial nephritis, stricture/obstruction, calcification
(24–50%), cavitating disease, with progression to renal failure. Calcification of
the kidney can be identifiable as renal or ureteric stone in up to 19% of cases. It
can mimic a neoplasm, as it can spread to outside the renal capsule and produce a
mass lesion.
CASE
A 73 year old Hispanic HIV-negative male with a history of recurrent urinary tract
infections and bilateral staghorn kidney stones was admitted for evaluation of weight
loss, fever and abdominal pain. He was found to have a pansensitive
Proteus mirabilis
UTI and was started on Levofloxacin.
A CT scan of abdomen showed bilateral staghorn calculi with obstruction and left hydronephrosis.
He underwent placement of left percutaneous nephrostomy tube from which pus also grew
pansensitive
Proteus mirabilis
. Renal scan then was performed, showing non-functioning left kidney. A decision to
do a left nephrectomy was made. Pathology of the left kidney showed extensive necrotizing
granulomatous nephritis with a few AFB and marked pyonephrosis. AFB culture of the
pus was negative.
With these findings, PPD was placed (was negative), sputum and urine AFB smear and
culture were negative. Chest X-ray showed bilateral apical pleural thickening and
a left pleural effusion which was tapped. About 400 cc of exudative fluid with lymphocytosis
was obtained. AFB culture and cytology were negative.
Started on tuberculosis treatment and the patient showed clinical improvement.
DISCUSSION
Although renal TB is an uncommon cause of progressive renal failure, it is potentially
preventable and easily treatable. Diagnosis depends on the physician considering the
possibility of TB and obtaining appropriate specimens for culture.
If TB is found early as the cause of obstruction, this may prevent unnecessary nephrectomy
or late complication of chronic TB infection that includes metaplasia that may be
a potential risk factor for squamous cell carcinoma.
RENAL TUBERCULOSIS AND CHRONIC RENAL INSUFFICIENCY: CHICKEN OR EGG FIRST?!
J. Schrader
1; J. Sheila1; B.L. Houghton1. 1Creighton University, Omaha, NE. (Tracking ID #116526)
LEARNING OBJECTIVES
Learning objectives: 1. Recognize that after lymphnodal involvement, most common form
of nonpulmonary tuberculosis is genitourinary disease. 2. Recognize that 26–75% of
renal tuberculosis coexists with active pulmonary tuberculosis. 3. Identify renal
tuberculosis as one of the rare causes of chronic renal insufficiency, and that chronic
renal insufficiency can predispose to renal tuberculosis.
CASE
Patient is a 57 year old Native American male who presented with generalized swelling
and progressive dyspnea of 4 months duration. He denied fever, chills, night sweats
or urinary symptoms. He had chronic dry cough, fatigue and weakness. Past medical
history included diabetes mellitus, hypertension, rheumatoid arthritis (on steroids)
and he was a recovering alcoholic. Approximately a year ago he was treated for culture
negative pyuria with antibiotics. He had history of positive PPD 10 years ago, untreated.
On admission, he was afebrile, and vital signs were stable. He had palor and significant
bilateral pitting edema. JVP was not elevated. Heart exam was unremarkable. Chest
exam revealed small bilateral pleural effusions and bibasilar rales. There was no
hepatosplenomegaly or free fluid in the abdomen. Initial labs showed hemoglobin 10.5
gm%, WBC 9.7, BUN 82 mg/dl and creatinine 3.3 mg/dl. Urinalysis was positive for protein
>500 mg/dl, WBC 25–50, RBC >100 and hyaline/fine granular casts. Pulmonary edema was
noted on Chest Xray. Ultrasound of the kidneys suggested intrinsic renal disease without
hydronephrosis. He was admitted, treated initially with diuretics. His renal failure
and dyspnea worsened requiring initiation of hemodialysis. Chest films showed worsening
infiltrates. Further workup showed positive AFB in sputum, urine and stool confirmed
as Mycobacterium tuberculosis. Rectal biopsy ruled out amyloidosis. He was started
on antituberculous therapy. Despite maximal therapy his condition continued to decline.
After detailed family discussion, it was decided to take patient home with hospice,
but continue ATT.
DISCUSSION
Genitourinary tuberculosis is the most common form of extrapulmonary tuberculosis
after lymphadenopathy. Renal involvement occurs through hematogenous spread from a
primary focus (most common being lungs) and is usually bilateral. Predisposing conditions
of renal tuberculosis include diabetes, chronic renal insufficiency, steroids and
alcoholism (all four present in our patient). Renal tuberculosis can present as sterile
pyuria, or as chronic interstitial nephritis (biopsy shows granuloma and caseation),
amyloidosis and end stage renal disease. Given the patient's treatment for sterile
pyuria in the past, it is uncertain if he had insidious onset of tuberculosis starting
then or whether chronic renal insufficiency predisposed to tuberculosis.
RETINAL HEMORRHAGES AND PAPILLOEDEMA IN PSEUDOTUMOR CEREBRI PRESENTING WITH SUDDEN
OF LOSS VISION
A. Devarajan
1; P. Patel1; A. Varadarajan1; E. Floranda1; C. Castillo1; C. Rathnakumar1; R. Jayachandran1.
1Jersey City Medical Center, Jersey City, NJ. (Tracking ID #116491)
LEARNING OBJECTIVES
A benign process affecting the brain which appears to be, but is not a tumor. It is
characterized by increased intracranial pressure and normal brain ventricle size.
There is no evidence of tumor, infection, and blocked drainage of the fluid surrounding
the brain or any other cause. The major symptoms of pseudotumor are increased pressure
within the skull (increased intracranial pressure—ICP). The cause for the condition
itself is unknown, and the diagnosis is made when other health conditions are ruled
out.
CASE
A 24 year old African American female with no toxic habits, and no known medical problems
except extreme obesity (BMI 44) presented with sudden loss of vision in both the eyes,
headache, nausea, and vomiting for one day. She denied dizziness, loss of consciousness,
seizure, photophobia, neck pain, tingling, numbness and weakness of extremities, Review
of other systems, family and social history were normal. She denies any medication
including oral contraceptive pills. Physical examinations including neurological examination
were normal except fundus showing bilateral papilloedema and retinal hemorrhage in
both eyes. Initial working diagnosis Pseudo tumor cerebri was made in view of the
sudden loss of vision in young obese female with no significant past medical history
including medications like oral contraceptive pills with benign neurological examination.
Initial CBCD and serum chemistry were normal. Lumbar puncture showed an opening pressure
of over 550 mm of water and a closing pressure of 210 mm of water. 15 cc of CSF was
removed during lumbar puncture. The CSF was clear, Glucose 80 mg, protein 20 mg, no
WBC and RBC. CSF stains and culture were negative for pathogens. Patient was started
on Acetazolamide. Patient relieved of her headache, nausea and vomiting and regained
partial vision after 24 hrs and complete vision in 6 days. Patient continued to have
residual papilloedema and hemorrhages in left eye.
DISCUSSION
Significance of clinical suspicion of Pseudotumor cerebri to be entertained in any
young obese female patients presenting with sudden loss of vision, nausea, vomiting,
and headache with no co-morbid conditions and no clinical evidence of any other causes.
Early institution of diagnostic and therapeutic lumbar puncture to relieve intra cranial
pressure and medical management with acetazolamide helps to prevent permanent visual
damage. Our case demonstrated clinical evidence of Pseudotumor cerebri with papilloedema,
and retinal hemorrhages and early diagnostic and therapeutic intervention restored
the vision within a few days, though the retinal changes remained unchanged for some
time.
RHABDOMYOLYSIS: AN UNUSUAL PRESENTATION OF HYPOTHYROIDISM
M. Derakhshani
1; J. Huang1. 1Louisiana State University Medical Center at Shreveport, Shreveport,
LA. (Tracking ID #115705)
LEARNING OBJECTIVES
1) Recognize rhabdomyolysis as a rare presentation of hypothyroidism. 2) Emphasize
the importance of history.
CASE
A 50 year old African-American female with no past medical history initially presented
to emergency room with a complaint of bilateral leg pain associated with lower back
pain progressively worsening for the past 3 months. Her symptoms did not respond to
a course of NSAIDS prescribed for “arthritis” by her local primary care physician.
She described the pain as dull, primarily in the lower back and both legs, constant
throughout the day and not related to physical activity. She reported ambulating and
performing activities of daily life without difficulty. She also denied any recent
history of vigorous exercise, over-ingestion of alcohol, or use of cocaine. Upon further
history, she admitted to cold intolerance, easy fatigue, and 20 lb weight gain within
the past 3–4 months. Her physical exam was unremarkable except for decreased deep
tendon reflexes. Musculoskeletal exam revealed normal tone, strength, and bulk in
all muscle groups. Laboratory data were significant for normal electrolytes, normal
renal function, elevated AST of 145 U/L (14–36) and ALT of 63 U/L (9–52) with normal
alkaline phosphotase of 46 U/L (38–126), decreased hematocrit of 31.4% (34–46), and
elevated serum creatine phosphokinase (CPK) of 16,000 U/L (30–135). Urine analysis
showed large blood on dipstick with only 10–20 RBC/HP suggesting myoglobinuria. Serum
myoglobin was elevated at 549 ng/ml (19–56). Further workup included ANA, viral hepatitis
panel, and urine drug screen which were all negative. Evaluation of thyroid function
revealed low T3 of 0.15 ng/ml (0.45–1.37), low T4 of 1.01 microg/dl (4.5–12.0), and
elevated TSH of >100 microU/ml (0.47–5.01) which confirmed the diagnosis of hypothyroidism.
After the initial intravenous fluid infusion, patient was followed in outpatient clinic
with thyroxin replacement. Her symptoms resolved and her CPK gradually normalized.
Her renal function remained normal throughout the course.
DISCUSSION
Muscle involvement in adults with hypothyroidism is common and includes stiffness,
myalgias, and mild weakness. However, overt rhabdomyolysis is quite rare with only
a few reported cases. The exact mechanism of rhabdomyolysis remains unclear. This
case suggests that rhabdomyolysis is a rare, but potentially serious complication
in hypothyroidism. It can be one of the initial presenting manifestations in undiagnosed
hypothyroidism. High index of suspicion and a thorough history, combined with readily
available thyroid function test, are essential for diagnosis. Earlier diagnosis in
this case might have prevented the indiscriminate use of NSAIDS that could have resulted
in renal dysfunction.
RHABDOMYOLYSIS AS A DRUG INTERACTION BETWEEN SIMVASTATIN AND NEFAZADONE: INCREASING
EVIDENCE
H.L. Korlakunta
1; S. Dodla1; R. Kizer1; S. Gonzalez1. 1Creighton University, Omaha, NE. (Tracking
ID #115719)
LEARNING OBJECTIVES
To recognize rhabdomyolysis as an adverse reaction of concurrent use of simvastatin
and nefazodone. We emphasize this by presenting, to our knowledge the fourth reported
case of myopathy or rhabdomyolysis caused by concurrent use of these drugs.
CASE
A 59-year-old white male was admitted to the hospital for sudden-onset of severe back
and lower extremity pain, weakness, and dark urine. He had no history of recent trauma
or prolonged heat exposure. He had a history of hyperlipidemia and depression, and
was taking simvastatin 80mg orally once daily and nefazodone 150 mg orally once daily.
Three weeks prior to admission, his simvastatin dose was increased from 40 mg to 80
mg orally once daily. On admission, laboratory studies revealed elevated creatine
kinase (CK) at 26,862 IU/L, elevated liver transaminases, elevation of serum creatinine
to nearly twice baseline, and urine sediment consistent with acute tubular necrosis.
Urine analysis demonstrated hematuria and positive urine myoglobin. Erythrocyte sedimentation
rate (ESR), antinuclear antibody (ANA), CMV and EBV serologies and thyroid stimulating
hormone were normal. White blood cell count was normal, and patient was afebrile.
Simvastatin and nefazodone were discontinued, and the patient was treated with IV
hydration. The patient's CK, liver transaminases, and urine sediment normalized with
resolution of his symptoms over the course of one week. He remained asymptomatic at
2 and 6-month follow-up examinations, and CK, liver transaminases, and serum creatinine
were normal.
DISCUSSION
Although myopathies and elevated liver transaminases are recognized as common side-effects
of HMG-CoA reductase inhibitors, susceptibility to rhabdomyolysis is significantly
increased by interactions with offenders such as cyclosporine, itraconazole, and some
macrolide antibiotics. These drugs inhibit the CYP3A4 enzyme family pathway, as does
nefazodone. This pathway metabolizes simvastatin. Inhibition of the CYP3A4 pathway
leads to increased levels of active statin metabolites, which increases the risk of
myopathies and rhabdomyolysis. The risk for rhabdomyolysis may be dose-dependent.
The increasing number of case reports of rhabdomyolysis associated with use of nefazodone
and simvastatin along with known interactions between simvastatin and other CYP3A4
inhibitors suggest that the combination should be avoided if possible. Though this
drug combination may be tolerated at certain doses, an increase in the simvastatin
dose may lead to rhabdomyolysis, as was demonstrated in the above case.
SARCOIDOSIS OR RIGHT HEART FAILURE? OR BOTH?
C. Leggett
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117483)
LEARNING OBJECTIVES
1. Recognize that the presenting feature of chest pain and shortness of breath in
a patient with sarcoidosis may represent worsening right heart failure.
CASE
A 37 year-old woman with a past medical history of sarcoidosis presented with worsening
shortness of breath and chest pain when walking only one block. She had associated
nausea and dizziness without syncope or diaphoresis; all symptoms were relieved by
rest. She had a right ventricular heave, but the remaining examination was normal.
Her CXR showed an enlarged cardiac silhouette and patchy infiltrates. An echocardiogram
revealed a pulmonary artery pressure of 70 mmHg with poor right ventricular function.
Left ventricular function was preserved. A chest CT confirmed stage III sarcoidosis.
She was started on prednisone. She was discharged with home oxygen and a diagnosis
of cor pulmonale secondary to her pulmonary sarcoidosis.
DISCUSSION
Physicians should consider the possibility of cardiac sarcoidosis in any patient with
a known history of sarcoid or in an otherwise healthy young person who develops arrhythmias,
conduction disease, or heart failure. A diagnosis of cardiac sarcoidosis is difficult
to diagnose without positive endomyocardial biopsy, and biopsy is only 20% sensitive
as lesions are often not confluent. There are several tests that can help to define
cardiac involvement. Patient's with granulomatous infiltration of the myocardium can
present solely with heart failure but there is also an increased incidence of arrythmias
or conduction abnormalities. An ECG or 24-hour holter monitor can detect if these
abnormalities are present. In a few patients with cor pulmonale, as suspected in this
patient, right ventricular hypertrophy regresses when the pulmonary disease is improved
with long term corticosteroids.
SATURDAY NIGHT FEVER
E. Chuong1; S. Shaw
1; N. Feldman1. 1UCLA/San Fernando Valley Program, Sylmar, CA. (Tracking ID #116965)
LEARNING OBJECTIVES
1. Identify fever of unknown origin (FUO) and common etiologies of FUO 2. Describe
a systematic diagnostic approach to FUO 3. Recognize a rare case of intravascular
lymphoma as a cause of FUO
CASE
A 47 year-old woman with hypertension and untreated latent tuberculosis (TB) presented
with 2 weeks of myalgia, arthralgia, weakness, fever, and confusion. Her physical
exam was significant for a temperature of 39.7°C, waxing and waning mental status,
and a II/VI systolic murmur. Initial laboratory data was notable for WBC 4,000 cells/uL,
hemoglobin 8 g/dL, platelets 29,000 cells/uL, unrevealing peripheral blood smear,
LDH 6046 U/L, ESR 60 mm/hr, D-dimer <5 ug/mL, fibrinogen 378 mg/dL, and troponin-I
21.8 ng/mL. Her chest radiograph suggested pulmonary edema, and she was intubated
for respiratory distress. CT and MRI scan of her head revealed ischemic changes; CT
scan of her chest indicated mutifocal pneumonia; CT scan of her abdomen and pelvis
demonstrated hepatomegaly with splenic infarct. Lumbar puncture, CSF studies, echocardiogram,
and bronchoscopy failed to demonstrate underlying disease. Bone marrow biopsy revealed
normocellular reactive tissue. The patient continued to be febrile throughout her
two-week hospitalization despite a negative evaluation for malignancy, hematological
disorder, infection, and collagen-vascular disease. Multiple studies were repeated,
but all remained undiagnostic. Broad-spectrum antibiotics, fluconazole, and anti-TB
medications were empirically started. The patient also received corticosteroids at
stress and pulse doses for presumptive collagen-vascular disease. Her critical illness
required multiple blood product transfusions and prohibited nuclear medicine scanning,
further biopsies, and exploratory laparotomy. The patient was diagnosed postmortem
with intravascular natural killer T-cell lymphoma.
DISCUSSION
Peterdorf and Beeson defined a fever of unknown origin in 1961 as a temperature greater
than 38.3°C on several occasions, illness duration longer than 3 weeks, and uncertain
diagnosis despite 1 week of inpatient investigation. Common causes of FUOs in the
adult population are malignancy, infection, and collagen-vascular disease. The evaluation
of an FUO begins with a detailed history and regular physical exams to identify probable
sources of FUO. Basic diagnostic testing should include complete blood count with
differential, serum chemistries and cultures, urinalysis and culture, applicable immune
and serological studies, tuberculin skin testing, and chest radiographs. Additional
serum testing, noninvasive imaging, radionucleotide studies, and invasive procedures
including biopsies may be helpful in the appropriate clinical context. Five to fifteen
percent of FUOs are undiagnosed despite exhaustive studies. Therapeutic trials of
antimicrobials and corticosteroids do not substitute for a carefully directed investigation
for the underlying cause of an FUO but may be warranted in cases of severe illness.
SAVING MY LIFE, BUT BREAKING MY HEART
B. Anderson
1; J. Wiese2. 1Tulane, New Orleans, LA; 2Tulane University, New Orleans, LA. (Tracking
ID #117386)
LEARNING OBJECTIVES
1. Recognize metabolic abnormalities associated with protease inhibitors. 2. Increase
awareness of the association between HIV-positivity and ischemic heart disease.
CASE
A 43 year-old man presented with one hour of left-sided chest pain that radiated to
the left arm. The chest pain occurred the day of admission while walking to work.
He was diagnosed with HIV fourteen years prior, and a history of untreated hyperlipidemia
that developed after instituting HIV therapy. He began treatment for HIV in 1995 with
a regimen including a protease inhibitor. His CD4 count and viral load two months
prior to admission were 504 cells/mm3 and <50 copies/mL. He denied any family history
of heart disease. His medications included efavirenz, lamivudine/zidovudine, and lopinavir/ritonavir.
His vital signs and physical exam was normal. An EKG revealed ST-segment elevation
in leads V2–V6. His initial serum troponin-I was 0.20 ng/ml. A percutaneous coronary
intervention was immediately performed, demonstrating a 70% stenosis of the proximal
left anterior descending artery. A stent was placed to the lesion with resultant TIMI
3 flow.
DISCUSSION
Metabolic abnormalities associated with protease inhibitors include hypertriglyceridemia,
hypercholesterolemia, and diabetes mellitus. As patients with HIV live longer, physicians
should be cognicsent of these side effects of anti-retroviral therapy that can lead
to accelerated atherosclerosis and increased myocardial risk. In this way, chronic
HIV infection is similar to diabetes as a comorbidity that predisposes to heart disease.
Because patients with HIV are increasingly living longer, coronary disease should
be aggressively pursued in this patient population.
SCLERITIS AS A PRESENTING MANIFESTATION OF WEGENER's GRANULOMATOSIS
Y. Ozawa
1; J. Blank1. 1UCLA—San Fernando Valley Program, Sylmar, CA. (Tracking ID #116296)
LEARNING OBJECTIVES
(1) Recognize scleritis as a manifestation of Wegener's Granulomatosis (WG). (2) Review
the various ocular and oral presenting symptoms of WG.
CASE
A 64 year old Guatemalan female presented to the ER with eye pain and decreasing visual
acuity for two months. One year ago she developed red, painful eyes which were treated
with antibiotics and cortisone in her country. Six months later, her eye condition
recurred resulting in bilateral corneal perforations. On review of systems in the
ER, she reported anorexia and a 30 pound weight loss over the previous 6 months and
recurrent rhinorrhea. She denied fever, cough, dyspnea, hemoptysis, joint pains or
hematuria. On exam, both sclerae were erythematous and her left eye had an opacified
cornea. A 3x1 cm ulcer was present on her lower anterior buccal mucosa with pronounced
erythematous gingiva and gingival hyperplasia. The rest of her exam was unremarkable.
Her labs were significant for a creatinine of 4.1 and urinalysis revealed microscopic
hematuria. A chest X-ray showed right upper lobe and right middle lobe cavitary lesions
as well as multiple left upper lobe nodules. A sinus CT demonstrated maxillary sinus
mucosal thickening. A C-ANCA (proteinase-3) was found to be elevated at 13. The presence
of three out of four clinical criteria (nasal/oral inflammation, abnormal chest radiograph
and abnormal urinary sediment) and the positive C-ANCA was considered diagnostic for
WG. Once infectious etiologies were ruled out, cyclophophamide was initiated with
gradual resolution of her symptoms.
DISCUSSION
WG is a systemic vasculitis of the small arteries and veins. It typically involves
the upper and lower respiratory tracts and the kidneys. The joints, eyes, mouth, skin,
heart, and nervous system may also become involved. Eye involvement (52% of patients)
may range from a mild conjunctivitis to proptosis, dacryocystitis, episcleritis, scleritis,
and corneal ulcerations. Over 50% of patients diagnosed with scleritis are ultimately
diagnosed with a connective tissue or vasculitic disease. Oral involvement (10% of
patients) in WG may include mucosal ulcerations, gingivitis and gingival hyperplasia.
Ulcerations are commonly found in the buccal mucosa and biopsy usually reveals necrotizing
vasculitis. Gingiva may be strikingly red with white, yellow, or blue punctate lesions,
clinically resembling over-ripe strawberries. Gingival biopsies reveal pseudoepitheliomatous
hyperplasia, multinucleated giant cells, and inflammatory infiltrates, a constellation
that is specific for WG. Most oral and ocular findings are nonspecific. However, scleritis,
which is a common manifestation of systemic disease, may alert the clinician to early
diagnosis and initiation of treatment.
SCLERODERMA UNMASKED: A CASE OF HYPERTENSIVE RENAL CRISIS
M. Hadian
1; B. Taqui1; N. Marchetti1. 1Temple University, Philadelphia, PA. (Tracking ID #116188)
LEARNING OBJECTIVES
1. Recognize the clinical manifestations of scleroderma and scleroderma renal crisis.
2. Recognize the importance of prompt treatment of scleroderma renal crisis. 3. Recognize
treatment options for scleroderma.
CASE
A 44 year old Hispanic male with hypertension presented with one day of headache,
chest pain, dyspnea and six months of progressive leg weakness, dysphagia and post-prandial
vomiting. Upon specific questioning, he also endorsed cold induced pain in his fingers.
Exam revealed a thin young man with minimal facial expression. His blood pressure
was 200/110. He had thickened facial skin and telangiectasia around nasolabial folds.
He had papilledema with AV nicking and an S4 gallop. He had purple atrophic fingertips
and thickened skin in the extremities. He had edematous lower extremities with 4–/5
proximal muscle strenth. Labs revealed BUN 42, Cr 5.7 (baseline 3.0), Hgb 9.5. His
cardiac enzymes were negative. His urine showed protein >300 mg/dl, 4–10 RBC. Intravenous
metoprolol, hydralazine and enalapril reduced his blood pressure to 160/85. Serologies
revealed a ANA 1/1280 with nuclear pattern, normal C3 and C4, negative anti-Scl 70.
Barium swallow revealed decreased motility. Sural nerve/quadriceps muscle biopsy showed
sclerodermal neuropathy and myopathy. Echocardiogram showed LV hypertrophy, EF 45%,
and increased pulmonary artery pressure. Subsequently, a kidney biopsy demonstrated
severe arteriolosclerosis suggestive of malignant hypertension, as well as focal segmental
glomerulosclerosis. The patient was diagnosed with scleroderma and discharged on enalapril,
prednisone and nifedipine.
DISCUSSION
Scleroderma is a disorder characterized by fibrosis of the skin, blood vessels and
visceral organs. Two major subsets are identified. The diffuse subset, characterized
by the rapid development of symmetric skin thickening of proximal and distal extremities,
face and trunk, is associated with visceral organ (heart, lung, kidney) involvement.
The limited cutaneous subset, involving distal extremities and face, is associated
with CREST syndrome (calcinosis, raynauds,esophageal dysmotility, sclerodactyly, telangiectasia).
Scleroderma renal crisis is defined by acute worsening of renal function and is usually
associated with abrupt onset of marked hypertension with retinopathy. Urine sediment
is usually normal, or with mild proteinuria. Blood pressure control is the mainstay
of therapy, with ace inhibitors as the agent of choice. Early recognition and treatment
of the crisis can preserve renal function. Our patient's creatinine returned to baseline
upon discharge. Renal crisis may unmask a diagnosis of scleroderma. Although there
is no cure, patients with scleroderma may benefit from d-penicillamine, steroids,
and calcium channel blockers.
SIMPLE HEART FAILURE OR SOMETHING MORE?
S.Y. Chien
1; J.H. Tillisch2. 1UCLA San Fernando Valley Program, Sylmar, CA; 2University of California,
Los Angeles, Los Angeles, CA. (Tracking ID #115658)
LEARNING OBJECTIVES
1. Recognize that the differential diagnosis of peripheral embolization and CHF symptoms
includes atrial myxoma. 2. Recognize imaging modalities necessary for the diagnosis
of cardiac tumors.
CASE
A 54-year-old woman was admitted with one month history of typical heart failure symptoms.
Echocardiogram showed diffuse global hypokinesis and EF 20%. Because the etiology
of her cardiomyopathy remained unclear, she was taken to cardiac catheterization.
It showed nonsignificant coronary stenosis. She responded to initial treatment with
diuretics. However, on additional questioning, she reported word-finding difficulty
and that her right leg had become more painful and cooler to touch for the past four
months. Eight months previously, she was found to have right renal artery occlusion
on ultrasound with elevated creatinine. A current work-up for possible stroke ensued,
including an MRI/MRA of the brain which demonstrated a recent ischemic infarction
in the right cerebellar hemisphere and posterior MCA territory. A doppler ultrasound
of the neck was negative. The stroke distribution suggested an embolic phenomenon,
but TTE was negative. Thus, an MRI of the heart was performed, which showed a small
round defect in the left atrium, with a bright rim. A TEE again confirmed a pedunculated
mass, attached near the interatrial septum with 1-cm stalk and 1-cm irregular spherical
portion, consistent with atrial myxoma. Irregular strands of swirling echoes were
also consistent with thrombus formation.
DISCUSSION
Primary cardiac tumors are exceedingly rare. Three quarters of them are benign, nearly
half are myxomas. Myxomas can occur in all age groups with a female predominence.
Sporadic cases are more frequent, but familial cases have also been reported. These
tumors usually develop in the atria, left more than right. Very rarely do they develop
in the ventricles. These tumors also have an array of interesting clinical manifestations
including systemic embolization involving the cerebral, retinal, renal, coronary,
pulmonary, and abdominal aorta circulations. Myxomas commonly give rise to obstruction
of cardiac filling. Signs of dyspnea, pulmonary edema, syncope, or sudden death may
occur, mimicking mitral or tricuspid-valve stenosis. Rarely does one find predominant
mitral or tricuspid insufficiency. Systolic or diastolic murmurs may be heard as well
as pericardial friction rubs. The first heart sound is often loud and widely split.
Laboratory findings might show anemia, thrombocytopenia, leukocytosis, and elevated
ESR or CRP. EKG findings and routine chest films are often nonspecific. The introduction
of echocardiography, CT, and MRI has greatly facilitated the diagnosis of cardiac
tumors. Treatment of choice is surgical resection. It is usually curative. In any
case, the systemic signs disappear after the tumor has been removed.
STENT PLACEMENT IN THE TREATMENT OF PULMONARY ARTERY STENOSIS SECONDARY TO FIBROSING
MEDIASTINITIS
R. Satpathy
1; C. Satpathy2; I.A. Khan1; V. Aguila1. 1Creighton University, Omaha, NE; 2SCB Medical
College, Cuttack, Orissa. (Tracking ID #115678)
LEARNING OBJECTIVES
Fibrosing mediastinitis is a rare benign disorder caused by proliferation of acellular
collagen and fibrous tissue within the mediastinum. Although many case are idiopathic,
many (and perhaps the most) cases in the United States are thought to be caused by
an abnormal immunologic response to Histoplasma Capsulatum infection. Affected patients
are typically young and present with signs and symptoms of obstruction or compression
of the superior vena cava, pulmonary veins or arteries, central airways, or esophagus.
Pulmonary artery (PA) stenosis is an infrequent complication of fibrosing mediastinitis.
CASE
We report a case of 41-year-old male who presented with fever. On examination he was
found to have cardiomegaly on chest x-ray and high ESR. The transthoracic echo (TTE)
showed pericardial effusion, which eventually resolved on its own. At that time, it
was thought to be viral in etiology. However, the patient continued to be symptomatic
in terms of chest pain, increasing fatigue, dizziness and progressively increasing
shortness of breadth with exercise over next 6 to 8 months. He had a repeat TTE done
showing supra pulmonic stenosis. A transesophagial echo was obtained and suggested
a mass compressing the pulmonary artery. The thoracic CT confirmed a 7 × 5 cm anterior
mediastinum mass, which on biopsy was found to be granulomatous with fibrosing mediastinitis.
AFB and fungal culture were negative. However, the serological test established the
diagnosis of histoplasmosis. He had a cardiac catheterization done afterwards, which
showed normal coronary arteries, SVC and pulmonary veins. His right heart pressures
included right ventricle (RV) 37/4, main PA 37/7, right PA 13/7 and left PA 23/7.
He had a large stent placed in the right pulmonary artery and post-stenting pressures
were RV 33/5, main PA 31/8 and right PA 26/8. He has been asymptomatic since then.
DISCUSSION
Despite varied forms of pharmacologic treatment and surgical interventions, most previously
reported patients with PA stenosis died of right heart failure as a result of severe
pulmonary hypertension. Although the placement of stents has been described as successful
treatment of congenital PA stenosis, there has been less description of PA stent placement
for fibrosing mediastinitis. This article describes a patient who was moderately symptomatic
from PA stenosis and has remained symptom-free for approximately 2 years now after
treatment.
STEROID USE IN GLOMERULONEPHRITIS ASSOCIATED WITH INFECTIOUS ENDOCARDITIS
M. Lim
1; C. Graeber2. 1University of Connecticut, Farmington, CT; 2New Britain General Hospital,
New Britain, CT. (Tracking ID #116110)
LEARNING OBJECTIVES
Recognize corticosteroid as an option in the management of renal dysfunction secondary
to glomerulonephritis associated with infectious endocarditis (IE) that does not improve
with appropriate antibiotic treatment.
CASE
A 66 year old Caucasian male with history of DM II, CHF, pacer for sick sinus syndrome
and atrial fibrillation presented with a 4 week history of night chills and 1 week
history of raised, tender, red lesions on all extremities. PE showed temperature of
102.6oF; 1/6 systolic murmur at the tricuspid area; and tender, palpable purpura diffusely
scattered over all four extremities, sparing the palms and soles. Laboratory showed
creatinine of 2.3 mg/dl (1.0 mg/dl 4 months PTA), BUN of 53 mg/dl; proteinuria (1.5
g/24 h) and hematuria. Serologies were as follows: ANA, RF, ANCA, anti-GBM, cryoglobulin
and HCVab were undetected. C3 and C4 were low. Blood cultures grew Streptococcus bovis.
2-D echo showed a 2 × 2 cm mass on the anterior leaflet of triculpid valve, sparing
the pacemaker. Colonoscopy disclosed diverticulosis and non-neoplastic colonic polyps.
The patient was treated with Ceftriaxone (2 g IV QD). Despite defervescence and sterile
blood cultures, his renal function deteriorated with creatinine reaching 5.3 mg/dl
on D22. Kidney and skin biopsy revealed focal necrotizing glomerulonephritis and perivascular
inflammatory changes, respectively. Prednisone (60 mg/d) was introduced (D22) and
creatinine dropped to 2.9 mg/dl on D29.
DISCUSSION
Glomerulonephritis complicates ∼20% of endocarditis cases. Patients usually present
with hematuria, red cell casts, variable degrees of hypertension and renal insufficiency.
This condition resolves with appropriate antibiotic treatment. However, as in the
case of this patient, a steady decline in renal function sometimes persists despite
apparent sterility of blood cultures, leading to death or ESRD. Plasmapharesis and
immunosuppressive therapy, alone and in combination have been reported in the literature
as management for these cases. There have been 8 reported cases in which corticosteroids,
along with antibiotics was shown to be effective in reversing azotemia, without compromising
treatment of endocarditis. The rationale for steroid use is thought to be the suppression
of immune reaction and immune complex formation since glomerulonephritis is believed
to be secondary to the deposition of immune complexes in the glomeruli and injuries
associated with this. This case report, in addition to the other reported cases in
the literature, suggests potential benefit from steroid use in IE-related glomerulonephritis
refractory to appropriate antibiotic treatment. The combination of rapidly progressive
glomerulonephritis and palapable purpura may be particularly common in IE due to Streptococcus
Bovis.
STREPTOCOCCUS AGALACTIAE PRESENTING AS A SARCOMATOUS MASS OF THE LOWER EXTREMITY
P.J. DiGiacomo
1; G. Sokos2. 1Allegheny General Hospital, Sewickley, PA; 2Allegheny General Hospital,
Pittsburgh, PA. (Tracking ID #117390)
LEARNING OBJECTIVES
To recognize the peripheral embolic manifestations of endocarditis. To recognize the
risk factors for Group B Steptococcal infections. To recognize a rare cause of endocarditis
in a healthy individual.
CASE
This is the case of a thirty-seven-year-old woman who presented with a painful mass
lesion of the right lower extremity. The lesion had developed over the course of two
to three weeks. On physical exam the patient had an eight centimeter by eight centimeter
firm tender mass of the posterior distal right lower extremity. The remainder of the
exam was normal. Computed tomography of the lesion suggested sarcoma or lymphoma as
the likely diagnosis. Prior to a surgical biopsy being performed the patient presented
to the emegency department with new onset seizure disorder. Evaluation of the patient
at this time revealed multiple bilateral mass lesions of the brain. Given the suggestion
of a more systemic process, cytology was performed on the lower extremity mass. This
revealed gram positive cocci. Given the findings the patient underwent echocardiography
revealing a large echogenic lesion of the aortic valve. Six sets of blood cultures
confirmed Streptococcus agalactiae as the causative organism. The periphreal embolic
manifestations in this patient included abscess of the lower extrmity, multiple brain
abscesses with new onset seizure, and an acute occlusion of the left superficial femoral
artery. Because of persistant embolic events the patient underwent an expidited aortic
valve replacement without further complication.
DISCUSSION
The common manifestations of endocarditis such as petechiae, Osler's nodes, Janeway
lesions, and splinter hemorrhages were not present in this patient. The more striking
large peripheral emboli created the morbidity in this patient. Streptococcus agalactiae
is a causative organism of adult infections in the peripartum period. Chorioamnionitis,
puerperal sepsis, endometritis, and urinary tract infection are the most frequent.
Our patient had an uncomplicated NSVD four months prior to presentation. This was
assumed to be the origin of her endocarditis. Risk factors for infecion at that time
are failure to screen for Group B streptococcal disease, failure to initiate intrapartum
prophylaxis, premature or prolonged rupture of membranes, prolonged labor, and delivery
at less than thirty-seven weeks of gestation. Historically these risks did not apply
to our patient. Risk factors for infection outside the genitourinary tract include
immunodeficiency states and common medical conditions such as diabetes. Our patient
had no such history. A history of acquired heart disease was also not present further
highlighting this as an unusual case of endocarditis in an otherwise healthy patient.
STREPTOCOCCUS INTERMEDIUS CAUSING RECURRENT PNEUMONIAS
B.P. Sankarapandian
1; S.K. Thambidorai1; M. Bandara1; M. Ricardo-Dukelow1; S. Dhanireddy2; L. Preheim1.
1Creighton University, Omaha, NE; 2Creighton University Medical School, Omaha, NE.
(Tracking ID #115701)
LEARNING OBJECTIVES
1.Recognize Streptococcus Intermedius (Strep Int.) as a potential cause for recurrent
pneumonias 2.Delineate risk factors associated with Strep Int. pulmonary infections
3.Emphasize the discordance between the clinical presentation and severity of illness.
CASE
57-year-old male with a history of alcoholism and smoking has symptoms of cough and
pleuritic chest pain. He had a history multiple pulmonary infections in the months
prior to admission. These infections were treated with several courses of flouroquinalone
and macrolide antibiotics. Despite the treatment the patient's condition did not improve
and he was eventually diagnosed at another facility with loculated empyema. He refused
inpatient therapy at that time and opted for continued outpatient therapy with oral
antibiotics. He subsequently presented to our facility with progressively worsening
dyspnea and pleuritic chest pain. Physical exam noted poor dentition with multiple
dental caries, absent breath sounds and dullness to percussion over the right lower
lung fields. Computerized tomography of the chest revealed right sided effusion with
multiple fluid filled loculations. Thoracentesis drained 2000 cubic centimeters of
exudative fluid. Pleural fluid cultures produced strains of Strep Int. The patient
was then treated with intravenous clindamycin and his condition rapidly improved.
DISCUSSION
Strep Int. is a rare but well reported pathogen in producing a variety of abscesses
in the body. There are several case reports identifying Strep Int. as a cause of pulmonary
infections. This patient presented with recurrent pneumonias that were treated with
standard antibiotics used for community acquired pneumonia. The patient subsequently
developed empyema allowing us to identify the organism responsible for the recurrent
infections. Appropriate treatment of pulmonary infections caused by Strep Int. will
include the addition of antibiotics with anaerobic coverage. Physicians must be cognizant
of the fact that patients with periodontal disease and alcoholism are susceptible
to Strep Int. infection. Respiratory infections with Strep Int. are characterized
by mild symptoms (without toxic features), the presence of predisposing factors (i.e.,
periodontal disease, alcoholism), thoracic empyemas and prolonged hospitalizations.
STRUMA OVARII PRESENTING WITH ASCITES AND AN ELEVATED CA-125 IN A PATIENT WITH KNOWN
GOITER
N.M. Dookeran
1. 1Boston Medical Center, Boston, MA. (Tracking ID #117524)
LEARNING OBJECTIVES
1) Recognize the variability in clinical features manifested by struma ovarii tumors.
2) Recognize that an elevated CA-125 level is not entirely specific to ovarian cancer.
CASE
This is a case of a 67-year-old woman with a history of chronic atrial fibrillation,
mitral regurgitation and right heart failure who was admitted with complaints of four
months of increasing abdominal girth, not responsive as in the past to her usual diuretic.
She had also recently been diagnosed with hyperthyroidism secondary to a multi-nodular
goiter and was being treated with methimazole. Physical exam revealed a cachectic
woman with a small, palpable left thyroid nodule, no jugular venous pressure elevation,
a heart exam consistent with atrial fibrillation and mitral regurgitation, and a normal
lung exam. Her abdomen was distended and non-tender, with shifting dullness and a
fluid wave. Her labs were significant for marked thrombocytosis, microcytic anemia
and hyponatremia. TSH and liver function tests were normal except for an elevated
INR due to warfarin use. Chest X-ray was normal. Abdominal CT scan revealed ascites,
a 7.1 × 5.3 cm complex left ovarian mass and serosal thickening of the stomach, colon,
omentum and gallbladder. The patient's CA-125 level was markedly elevated at 889 units/ml.
However, ascites cytology was negative for tumor cells and the patient eventually
underwent an exploratory laparotomy and left salpingo-oophorectomy. Pathology revealed
a non-functional struma ovarii.
DISCUSSION
Struma Ovarii, a rare cystic ovarian teratoma, consists mainly of thyroid tissue and
can vary widely in presentation—from being asymptomatic to having ascites and possibly
pseudo-Meigs' syndrome. The current case, prior to surgery, was concerning for ovarian
cancer. There have been few similar cases of struma ovarii presenting with an elevated
CA-125 level. In addition, the co-existence of a multi-nodular goiter made this patient's
case more unique. This, along with reports of struma ovarii occurring more often in
countries where goiter is endemic, raises the question of whether there are genetic
and/or environmental factors that predispose to both conditions.
SWEET's SYNDROME
O.F. Osi-Ogbu
1; R. Granieri1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115885)
LEARNING OBJECTIVES
1 To recognize the dermatologic manifestations of Sweet's syndrome 2 To recognize
the potential association of Sweet's syndrome with malignancy 3 To recognize the essential
role of skin biopsies in uncommon dermatosis
CASE
A 43 year old previously healthy male presented with a diffuse, vesicular, painful
rash and fever of a day's duration. One month earlier, he was treated for an upper
respiratory tract infection with azithromycin. Physical examination revealed a temperature
of 38.2°(C), multiple erythematous 10–40 mm papulovesicular lesions, predominantly
on his neck, shoulders, trunk, face and extremities. Laboratory studies revealed WBC
11.5 (85% neutrophils). The initial impression was disseminated herpes vs Sweet's
syndrome. Culture was negative for herpes. A punch biopsy revealed neutrophilic infiltrates
consistent with Sweet's syndrome. Chest CT showed a left lower lobe thyroid nodule.
FNA was negative for malignancy.
DISCUSSION
Originally described in 1964 by Robert Sweet, Sweet's syndrome is characterized by
the abrupt onset of tender, red to purple circinate plaques. The syndrome ranges from
this classic presentation, occurring in young women after mild respiratory illness,
to a more aggressive neutrophilic process that may be associated with inflammatory
diseases or malignancy. Massive epidermal edema may produce a deceptively vesicular
appearance. Ulcers and bullae are more common in malignancy associated disease. Sweet's
syndrome demonstrates pathergy. Although the skin is the primary target organ, extracutaneous
manifestations occur and include pulmonary infiltrates, proteinuria, hematuria and
decreased GFR. The female to male ratio is 2–3:1. Several malignant and nonmalignant
disease have been associated with Sweet's syndrome, including myelodysplasia, CML,
AML, lymphoma, malignancy of the genital tract and upper respiratory infections. The
presence of 2 major and 2 minor clinical findings are criteria for diagnosis. Major
criteria include (1) abrupt onset of tender or painful erythematous plaques or nodules,
occasionally with vesicles, pustules, or bullae and (2) predominant neutrophilic dermal
infiltrates without leukocytoclastic vasculitis. The minor criteria are (1) antecedent
respiratory or GI tract infection, vaccination or associated inflammatory disease,
hemoproliferative disorders, solid malignant tumor or pregnancy (2) malaise and fever
>38 (3) ESR > 20 mm, elevated C-reactive protein, segmented neutrophils and bands
>70 %, leukocytosis > 8000, (4) excellent response to systemic corticosteroids. Skin
biopsy is the main stay of diagnosis. Medical management includes prednisone (40–80
mg qd). Prognosis depends on the underlying cause.
SYMPTOMATIC HYPOMAGNESEMIA AND HYPOKALEMIA: A CASE OF GITELMAN's SYNDROME COMPLICATING
A THIRD TRIMESTER PREGNANCY
J. Baez-Escudero
1; A. Samuels1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116184)
LEARNING OBJECTIVES
1. Recognize clinical manifestations of Gitelman's Syndrome. 2. Learn to differentiate
Bartter's Syndrome from Gitelman's Syndrome. 3. Recognize possible complications of
severe electrolyte disturbances during late pregnancy.
CASE
A 26 year old G1P0A0 Hispanic female with childhood Bartter's syndrome presented in
her 32nd week of gestation with severe premature uterine contractions and threatened
preterm labor. Prior to her pregnancy she was chronically hypokalemic and mildly alkalotic,
but was well controlled with potassium supplementation and spironolactone. Spironolactone
was discontinued during pregnancy. On admission, she had increased uterine activity
and contraction frequency with mild changes in fetal heart rate. She had minimal cervical
dilatation and effacement. Urinary and vaginal infections and other triggers were
excluded. Her serum electrolytes were: Na 137, K 2.6, Cl 104, Ca 9.8, magnesium 0.4,
phosphorus 3.2. Her serum pH was 7.45, and creatinine 0.8 mg/dL. After rapid intravenous
repletion of magnesium and potassium, her contractions ceased. She contined to require
electrolyte repletion and was discharged four days later. Prior to her pregnancy,
she had not been severely hypomagnesemic. Two weeks later she delivered a premature
34 week old infant who had no electrolyte disturbances. The patient now requires both
oral potassium and magnesium to avoid other systemic manifestations. A diagnosis of
Gitelman's syndrome was clinically confirmed.
DISCUSSION
Classic Bartter's syndrome is an autosomal recessive disorder characterized by sodium
wasting, hypokalemic metabolic alkalosis, hyperreninemic hyperaldosteronism with normal
or reduced blood pressure, urinary concentrating defect, and hypercalciuria. It usually
presents in infancy or early childhood. Gitelman's syndrome is a variant of Bartter's
syndrome that is distinguished primarily by hypocalciuria and hypomagnesemia. Patients
with Gitelman's syndrome usually present later in life (after age 6) and have milder
symptoms. The defect is due to inactivating mutations in the distal tubule sodium-chloride
cotransporter. Hypomagnesemia due to renal wasting is universally found in patients
with Gitelman's syndrome. Women of childbearing age with this disorder are at risk
for preterm delivery secondary to severe hypomagnesemia and hypokalemia, well known
triggers for premature uterine contractions. It is unclear whether pregnancy exacerbates
electrolyte wasting. Treatment includes potassium and magnesium supplementation as
well as spironolactone.
TB OR NOT TB? THAT IS THE QUESTION
H. Ly
1; M. Rotblatt2. 1University of California, Los Angeles, Sylmar, CA; 2UCLA SFVP-Olive
View Medical Center Department of Internal Medicine, Sylmar, CA. (Tracking ID #115714)
LEARNING OBJECTIVES
1. To recognize the presentation of Typhoid Fever. 2. To recognize the importance
of this illness in undeveloped countries and returning travelers.
CASE
A 49 year old Hispanic male without significant medical history who returned from
a 2 month field job in Mexico complaining of intermittent fevers and headache for
3 weeks. He also reported nightsweats for 1 week and a dry cough for 2 days. He had
additionally lost 10 lbs from poor appetite over the past month. There were no GI
complaints. He denied any sick contacts, TB exposure, unusual foods, smoking, or IV
drug use. He did work near cattle, even witnessing the birth of a calf. His vital
signs were T 38.9 C, BP 100/65, P 98, R 18, and O2 sat 98% on room air. He was diaphoretic
and warm to touch on exam, but otherwise, his remaining physical examination was unremarkable.
His WBC was 4.3 (N 83, L 14, M 3), Hb 12.1, Hct 35.8, and Plt 245. His chemistry panel
was normal. His liver enzymes were: ALT 112, AST 97, Alk Phos 97, Tot bili 0.7. Chest
X-ray was unremarkable. He was initially admitted to rule out TB and further evaluate
this ill-defined illness. Considering his travel and exposure history, our differential
included TB, community acquired pneumonia (CAP), malaria, hepatitis, brucellosis,
and coccidiomycosis. Lymphoma was also considered. A battery of cultures and tests
were sent. Meanwhile, he was isolated for TB and empirically treated with ceftriaxone
for CAP. Over the next few days, he began to defervesce. Interestingly, he had a relative
bradycardia for temperatures averaging over 39 C. His blood culture eventually grew
Salmonella typhi, and his antibiotic was switched to levoquin. Within the next few
days, he was discharged after full defervescence with marked improvement in symptoms
and normalizing liver enzymes. His other tests were negative for TB, malaria, hepatitis,
HIV, brucellosis, coccidiomycosis, and lymphoma.
DISCUSSION
Our case of Typhoid Fever exemplified the difficulty in a clinical diagnosis given
its non-specific presentation. The symptomatology in our patient closely resembles
the classic textbook description. Although our patient did not have the classic rose
spot rash or hepatosplenomegaly, he did have relative bradycardia. Treatment of choice
is a fluoroquinolone, but 3rd generation cephalosporins are also effective. While
no longer common in developed countries, Typhoid fever is still prevalent in undeveloped
countries where sanitation remains poor. Like TB, it is important to include in the
differential diagnoses of unexplained fevers in travelers.
THE BLOOD CULTURE THAT ROTATED OFF SERVICE
S. Shaw
1; M. Rotblatt1. 1UCLA/San Fernando Valley Program, Sylmar, CA. (Tracking ID #117005)
LEARNING OBJECTIVES
1) Recognize the prolonged diagnostic course for endocarditis due to HACEK organisms.
2) Recognize that discharging patients when all team members rotate off service can
be hazardous. 3) Recognize the need for hospitals to have a back-up plan to follow-up
positive blood cultures.
CASE
A 43 year old man with a history of DM presented to the ED complaining of left shoulder
pain for two days after a fall. Vital signs were T 38.1, BP 102/42, RR 29, and HR
129. Physical exam was significant for rigors, right basilar rales, a 2/6 systolic
murmur at the LUSB, 1+ pitting LEE, and generalized tenderness of the left shoulder.
Labs were significant for WBC 18.6, Hb 6.2, Hct 17.9, Na 123, K 5.8, Cl 90, HCO3 21,
Cr 1.1, glucose 503, and urinalysis with pH 5.0, 4 WBC and 5 RBC. The CXR demonstrated
right hilar fullness. He was thought to have a UTI, a possible pneumonia, anemia of
chronic disease based on previous workup, dehydration, possible type 4 RTA, and possible
adrenal insufficiency…that is, until a TTE revealed a 2 × 2 cm vegetation on the right
coronary cusp of the aortic valve with associated severe aortic insufficiency. Review
of past laboratory results discovered a blood culture positive for Haemophilus aphrophilus
taken two months earlier during hospitalization for new-onset diabetes, fever and
elevated WBC. Repeat physical exam revealed embolic lesions on his toes and JVP 15
cm after hydration. Brain MRI showed evidence of septic emboli. He was diagnosed with
infective endocarditis (IE) with embolic phenomena. He underwent valve replacement
surgery and did well. On review of the previous admission, it was noted that the patient
had been discharged at the end of June with blood cultures negative after 5 days;
when the positive culture at 21 days was filed, the resident had graduated, the rotating
intern had returned to his home hospital, and the attending had switched.
DISCUSSION
Prompt diagnosis of infective endocarditis (IE) is complicated by its myriad presentations.
Of note, younger patients may present with heart failure with no prior cardiac disease;
musculoskeletal complaints may be an early symptom in up to 40% of patients; and most
patients with IE have an abnormal urinalysis. Furthermore, in 2-5% of patients with
IE, no organism is isolated after three serial blood cultures. In cases with negative
cultures, if there is high clinical suspicion, the HACEK organisms (H. aphrophilus,
H. parainfluenzae, Actinobacillus actinomycetemcomitans, Cardiobacterium hominis,
Eikenella sp., and Kingella sp.) should be suspected. However, blood cultures may
need to be incubated for prolonged periods. In academic centers with multiple rotating
team members, there is often no straightforward follow-up for results that become
available after all members are off service. In some centers, the Infectious Disease
service takes responsibility. Each hospital should have a plan in place to follow
up positive cultures.
THE CASE OF THE LITTLE OLD LADY WHO WASN'T QUITE SWEET ENOUGH
J. Beversdorf
1; J.L. Sebastian1; D. Torre1. 1Medical College of Wisconsin, Milwaukee, WI. (Tracking
ID #116777)
LEARNING OBJECTIVES
1) To recognize that insulinomas can present as a new diagnosis in elderly patients
and 2) to recognize insulinoma as a cause of hypoglycemia even when sophisticated
pancreatic imaging studies are normal.
CASE
An 82-year-old non-diabetic woman, a retired registered nurse, presented to the emergency
room with symptoms of dizziness, cold sweats and visual changes. She was found to
have a blood sugar of 42 mg/dl and administration of one amp of D50 promptly relieved
her symptoms. The patient reported that she had experienced similar symptoms since
1998 and that her previous physician had prescribed treatment with prednisone to alleviate
these spells. During the past year, her symptoms had increased in both frequency and
severity. Upon admission to the hospital, the patient's vital signs and physical examination
were essentially unremarkable. Initial laboratory studies revealed that the following
tests were normal: urinalysis, blood urea nitrogen, serum creatinine and liver enzymes.
Insulin and C-peptide levels were both elevated, glycated hemoglobin level was 5.5
and a sulfonylurea screen was negative. An abdominal MRI and endoscopic ultrasound
to evaluate for the presence of an insulinoma did not reveal any pancreatic lesions.
Early during the patient's hospital stay, she remained asymptomatic and her blood
sugars ranged between 70 to 130 mg/dl. One morning, the patient suddenly experienced
symptoms of sweating and palpitations. Her blood glucose dropped to 26 mg/dl and she
became unresponsive. The patient promptly regained consciousness after receiving two
amps of D50 and a continuous infusion of D10. Following this episode, the patient
was scheduled for an exploratory laparotomy at which time a 1.6 cm lesion on the tail
of the pancreas was found and removed. Histopathology revealed findings compatible
with an insulin-secreting islet-cell tumor.
DISCUSSION
Severe hypoglycemia in the absence of diabetes, alcohol, exogenous administration
of insulin or use of drugs which stimulate endogenous insulin secretion is thought
to be quite uncommon. In the setting of spontaneous hypoglycemia, the diagnosis of
insulinoma is highly suggested by the finding of elevated plasma insulin and C-peptide
levels. Once an insulinoma is suspected, it is important to identify the tumor preoperatively
as localization at the time of surgery may be quite difficult. Although the test characteristics
of pancreatic imaging studies vary from institution to institution, the sensitivity
of endoscopic ultrasound has been reported to be as high as 93%. This case emphasizes
that clinical acumen and a high index of suspicion remain crucial to making an accurate
diagnosis.
THE CASE OF THE STUBBORN SWOLLEN LEG
L.A. Blauwet
1; A.K. Ghosh1. 1Mayo Clinic College of Medicine, Rochester, MN. (Tracking ID #115041)
LEARNING OBJECTIVES
1. Recognize May Thurner syndrome as an unusual etiology for deep venous thrombosis
(DVT). 2. Discuss the approach to diagnosis and treatment of May Thurner syndrome.
CASE
A 71 year-old woman, status post repair of a left open tibiofibular fracture nine
weeks previously, presented with a 2-day history of left lower extremity discoloration,
pain and swelling. A duplex Doppler ultrasound revealed thrombus extending from the
left external iliac vein proximally to the left femoral and popliteal veins distally.
Physical examination revealed an extremely swollen and tender left leg that was dusky
purple from the ankle to the groin. Pedal pulses were not palpable but were present
using Doppler ultrasound. Unfractionated Heparin and Warfarin were initiated, and
the patient was given narcotics for pain relief. Despite this, the patient's left
leg showed no signs of improvement the following two days. Interventional radiology
was then consulted. An inferior vena cavogram revealed findings suggestive of May-Thurner
syndrome. Successful mechanical thrombectomy was performed and Urokinase was infused
regionally. Follow-up venogram the next day revealed severe narrowing of the left
common iliac vein and the left upper external iliac vein. Balloon dilation was performed
and then a Wallstent was deployed in the external iliac vein. Symptoms quickly resolved.
Plavix was given for one month, and then low dose aspirin was initiated. Warfarin
was continued, with a target INR of 2.0–3.0. Hormone therapy was discontinued. Ongoing
stent patency will be assessed by serial duplex Doppler sonography. Duration of anticoagulation
treatment will be determined based upon sonographic findings.
DISCUSSION
May-Thurner syndrome (i.e., iliac compression syndrome) is the development of acute
iliofemoral DVT by compression of the left common iliac vein against the spine and
pelvic brim by the right common iliac artery. It occurs most commonly in 20–40 year-old
women. Diagnostic ascending venography is essential to accurate diagnosis. Definitive
treatment includes mechanical thrombectomy, catheter-directed thrombolytic therapy,
and stent placement in the left common or external iliac vein. In our patient, additional
risk factors for DVT included a 20-year history of hormone therapy and recent fracture.
Poor resolution of leg swelling compelled us to look for an alternative etiology of
DVT and seek thrombolytic therapy in this case.
THE CRIMSON LUNG: A CASE OF WEGENER's GRANULOMATOSIS
G. Agarwal
1; R. Granieri1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115119)
LEARNING OBJECTIVES
1) Recognize the presentation of Wegener's Granulomatosis (WG) 2) Diagnose WG using
clinical, laboratory, and histologic parameters 3) Recognize the treatment of WG
CASE
E.D. is a 23 y/o WM who was well until he noted diffuse myalgias and arthralgias two
months prior to presentation. He subsequently developed discomfort and redness in
both eyes and immeditaley prior to presentation, he noted a sore throat, rhinorrhea,
and anorexia followed by dyspnea on exertion, fevers, and hemoptysis. He had no significant
past medical history and was taking no medications. He was a ten pack year tobacco
smoker and occasionally used cocaine and marijuana. Upon presentation, he was in respiratory
distress and was intubated. His exam was notable for a temperature of 38.2(C), heart
rate of 112, and respiratory rate of 22. He had diffuse coarse breath sounds but an
otherwise unremarkable exam. His labs were notable for elevated creatinine, normochromic
normocytic anemia, mild leukocytosis, markedly elevated sedimentation rate, and hematuria.
A C-ANCA titer was 1:1280. The patient underwent a VATS procedure with biopsies revealing
a granulomatous vasculitis consistent with Wegener's Granulomatosis (WG). He was begun
on pulse IV steroids and cyclophosphamide, and subsequently tolerated extubation.
DISCUSSION
WG is a vasculitis of medium and small arteries that primarily involves the respiratory
tracts and kidneys. Presenting symptoms include purulent/bloody nasal discharge, oral
and/or nasal ulcers, polyarthralgias, and myalgias. Renal disease is common, being
manifested by acute renal failure, hematuria, and proteinuria. The American College
of Rheumatology proposed diagnostic criteria for WG (formulated prior to the availability
of antineutrophil cytoplasmic antibody (ANCA) testing) which inlcude nasal/oral inflammation,
abnormal CXR, abnormal urinary sediment, and granulomatous inflammation on biopsy
of an artery. Two or more of these criteria yielded a sensitivity of 88% and a specificity
of 92%. The diagnosis of WG is also suggested from circulating ANCA that are usually
directed against proteinase 3 (C-ANCA). Nearly all patients with active WG have circulating
ANCA. However, ANCA alone, including C-ANCA which is more specific for WG, does not
appear to be sufficiently accurate to establish the diagnosis. The diagnosis is confirmed
by tissue biopsy. Granulomatous inflammation and frank vasculitis are potential biopsy
findings. Daily oral cyclophosphamide-corticosteroid therapy is the initial favored
treatment. Once remission is induced, alternative regimens including methotrexate
and azathioprine have been employed. Survival in untreated WG is poor, with up to
90% of patients dying within two years. Survival has significantly improved with the
introduction of cyclophosphamide-corticosteroid therapy.
THE MAN WITH THE SWOLLEN LEG-A LESSON FROM A PRIMARY CARE HIV CLINIC IN UGANDA
A.E. Torreblanca
1. 1University of California, San Francisco, San Francisco, CA. (Tracking ID #116547)
LEARNING OBJECTIVES
1) Recognize the causes of secondary lymphedema 2) Recognize Kaposi's sarcoma (KS)
as a leading cause of cancer in sub-Saharan Africans 3) Recognize treatment limitations
in sub-Saharan Africa.
CASE
A 42 year old male presented to the Reach Out Clinic in Kampala, Uganda complaining
of swelling in his right leg. He first noted dark lesions on his feet 2 years prior
to presentation. These lesions gradually spread to his groin and were associated with
leg swelling. He did not know his HIV status but reported having 2 wives, one of whom
died 2 years ago of tuberculosis. Examination of his right leg revealed non-pitting
edema, numerous purple papules, and palpable inguinal nodes bilaterally. Serology
was positive for HIV. He was sent to a public hospital where skin biopsy showed Kaposi's
sarcoma. He was started on bleomycin and vincristine with minimal improvement. The
clinic is currently trying to find a sponsor to pay for his antiretroviral therapy.
DISCUSSION
Non-pitting edema is generally due to lymphedema. Causes of secondary lymphedema includes
lymph node trauma (surgery, radiation), malignancy (pelvic, KS), and infection (filariasis).
KS is a common cause of lymphedema in sub-Saharan Africa. The four forms of KS are
classic, endemic-African, organ transplant-associated and AIDS-related. In the era
of AIDS there has been a 20-fold increase in the occurrence of KS in Uganda. Review
of the cancer registry in Kampala from 1989–91 reported KS to be the leading cancer
in males (48.6%) and the second most frequent (17.9%) in females. Treatment options
for KS include local therapy (radiation, intralesional chemotherapy), and systemic
chemotherapy (liposomal anthracyclines, bleomycin and vincristine). Although chemotherapy
has proven effective in endemic-African KS, its effect on AIDS-related KS is limited.
The widespread use of antiretrovirals (ARVs) in the western world has lead to a marked
decline in new AIDS-related KS. In Uganda ARVs are only available to patients who
can pay for the drugs or qualify for clinical trials. Patients with advanced KS are
currently ineligible for the clinical trials underway in Uganda. The typical ARV regimen
is Triommune (a generic combination of d4T/3TC/NVP). This regimen costs ∼$26 per month;
the typical Ugandan lives on less than $30 a month.
Lymphedema secondary to Kaposi's sarcoma.
THE MARDI GRAS HEART SYNDROME
L. Quan
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117502)
LEARNING OBJECTIVES
1. To recognize the clinical presentation of atrial fibrillation in the holiday heart
syndrome. 2. To emphasize the treatment of atrial fibrillation. 3. To identify the
indications and contraindications for anticoagulation in atrial fibrillation.
CASE
A 29 year-old man presented with palpitations. He denied chest pain, dyspnea, or any
other associated symptoms. During his vacation to New Orleans, he went on a binge
of over twenty cans of beer. His cardiac enzymes were negative and his TSH was normal.
An EKG showed atrial fibrillation with PVC's and a heart rate of 112. The heart rate
was controlled with diltiazem. He was ruled out for cardiac thrombosis with a TEE
and subsequently successfully cardioverted to a normal rhythm. Because he refused
coumadin, he was discharged on aspirin.
DISCUSSION
Moderate alcohol consumtion (7 to 11 drinks per week) is associated with decreased
cardiovascular mortality. Consuming all eleven drinks on the same occasion, however,
may lead to an alcohol hangover associated with increased adrenergic tone, myocardial
work, and cardiovascular morbidity. Atrial fibrillation is the most common arrhythmia
associated with the hangover period, found in up to 60% of binge drinkers with or
without underlying alcoholic cardiomyopathy. Treatment consists of rate control with
calcium channel blockers, beta blockers, or digoxin. Recurrent or persistent atrial
fibrillation may necessitate anti-arrythmic medications or cardioversion, and long-term
anticoagulation. In addition to arrhythmias, the alcohol hangover is associated with
an increased c-reactive peptide and thromboxane B2, both markers of systemic inflammation.
This inflammation, in concert with increased myocardial work, may explain the two-fold
increased risk of myocardial infarction in patients who are frequently hungover. Physicians
should advise their patients that there are unique harms associated with the alcohol
hangover.
THE CASE OF THE FEVERISH FRENZY
C. Weaver
1; J. Hefner1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116221)
LEARNING OBJECTIVES
1. Recognize a common presentation of Familial Mediterranean Fever (FMF) in an uncommon
patient; 2. Diagnose, manage and treat FMF; 3. Recognize validation of a patient's
symptoms is often of therapeutic significance.
CASE
A previously healthy 28 year-old female of Italian and Anglo-Saxon descent, presented
to the outpatient setting complaining of intermittent fevers (37.8 to 39.5) and abdominal
pain over 3 years. The patient frequently presented to the ED and was hospitalized
5 times. Emergent cholecystectomy and appendectomy were performed, with normal pathology.
Six CT scans, one MRI, an angiogram, cystoscopy, CBC's, LFT's, blood and urine cultures,
viral titers, lipase, porphyria evaluation, heavy metals, ANA, ds-DNA, complement
levels and lipids were non-diagnostic. The only abnormalities were microscopic hematuria
and beta-thalessemia minor. There was no pattern to the attacks and no exacerbating
features. She had failed over-the-counter therapies and was taking no medications.
She was well between attacks initially but became depressed over the past year. The
frequency and severity of attacks and absence at work forced her to leave her job.
She had been referred to psychiatric services on numerous occasions. Physical exam
was unremarkable. Based on history, colchicine was started and she was instructed
to obtain tests only during an attack. Several months later, after a tremendous response
to colchicine, she had elevated LFT's, ESR, CRP and a leukocytosis upon presentation
with abdominal pain and fever to 39.1.
DISCUSSION
FMF is an autosomal recessive condition manifesting in paroxysms of fever and severe
abdominal pain. Primarily seen in persons of Mediterranean descent, it is also found
in other groups, including Anglo-Saxons. Ten percent of patients present after the
age of 20. The differential diagnosis includes acute surgical abdomen, acute intermittent
porphyria, vasculitides/SLE, and relapsing pancreatitis/hypertriglyceridemia. Colchicine
is the mainstay of treatment. A study published in 1991 determined that 72% of patients
on colchicine averaged 1 attack in 6 months. Colchicine decreases the occurrence of
amyloidosis, a serious consequence. FMF is primarily a clinical diagnosis; a reasonable
evaluation includes a trial of colchicine and measurement of ESR, haptoglobin, CRP,
and fibrinogen during an attack. FMF can be the cause of numerous ER visits, extensive
laboratory testing and tremendous frustration for both patient and caregiver. High
clinical suspicion is necessary and a PCP should handle management for optimal coordination
of care. Validation of a patient's symptoms and distress can be of therapeutic significance
and provide comfort in the setting of chronic disease.
THE SHOCKING CONSEQUENCE OF DISCONTINUING PHENYTOIN
B. Lee
1; M. Rotblatt2. 1UCLA-SFVP, Sylmar, CA; 2UCLA/San Fernando Valley Program, Sylmar,
CA. (Tracking ID #115654)
LEARNING OBJECTIVES
1) To recognize antiarrhythmic properties of phenytoin. 2) To review treatment of
recurrent ventricular tachycardia (VT).
CASE
A 68 year-old male presented to cardiology clinic with a complaint of multiple ICD
firings. He had a single-chamber ICD placed 9 years previously for sustained VT which
had been well controlled on chronic sotalol therapy with no ICD firings for the past
4 years. The patient denied chest pain, lightheadedness, or shortness of breath prior
to firings. On further questioning, the patient had been taking phenytoin for seizure
prophylaxis for an intracranial hemorrhage 4 years prior to admission, and had just
been tapered off 3 weeks prior. Despite an increase in his dose of sotalol from 80
mg QD to 120 mg BID, the patient continued to experience firings of his ICD. He was
subsequently restarted on phenytoin with complete resolution of ICD firings.
DISCUSSION
Sustained VT is defined as VT that persists for greater than 30 seconds or requires
termination because of hemodynamic collapse. VT generally accompanies structural heart
disease such as coronary artery disease, cardiomyopathies, right ventricular dysplasia,
valvular heart disease, and heart failure. VT may also occur in the absence of structural
heart disease as seen with metabolic disorders, Brugada syndrome, prolonged QT syndrome,
or idiopathic VT. The prevention of recurrent VT includes drug therapy (with or without
selection through programmed stimulation) as well as devices combining antitachycardia
pacing with ICD. In our patient, sotalol was started concomitantly with ICD placement
in 1994 and phenytoin was started several years later for seizure prophylaxis. Though
our patient had multiple risk factors for recurrent VT including a history of CAD
s/p 4 vessel CABG, critical stenosis of his mechanical aortic valve, and heart failure
(EF: 25%), the discontinuation of phenytoin appears to have caused the recurrent VT.
Phenytoin is used mainly in the prophylactic management of tonic-clonic seizures and
partial seizures with complex symptomatology. Moreover, phenytoin may be used for
the prevention and treatment of seizures occurring during neurosurgery and in the
treatment of status epilepticus. However, phenytoin is also a Class 1b antiarrhythmic,
and an unlabelled use is in the treatment and maintenance of VT and paroxysmal atrial
tachycardia, particularly in those patients who do not respond to conventional antiarrhythmic
agents or cardioversion. Although phenytoin was started for seizure prophylaxis in
our patient, it served a dual purpose as an antiarrhythmic as well. The VT was not
well controlled despite increasing the sotalol dosage, and phenytoin was ultimately
restarted with the desired effect.
THE UNFITTING PROSTHESIS
T. Tanabe
1. 1University of Pennsylvania, Philadelphia, PA. (Tracking ID #117100)
LEARNING OBJECTIVES
1) Recognize the importance of carfeful stump examination in diabetic patients. 2)
Suspect limb- or life-threatening infections early and facilitate the diagnostic workup
in diabetic patients.
CASE
A 63-year-old man presented with a pain at the left stump for three days. Past medical
history included chronic obstructive pulmonary disease, systolic dysfunction and diabetes.
The patient underwent below knee amputation of left leg in 1998. He noticed a dull
pain 3 days prior to presentation when he was walking on prosthesis, which he had
used for 4 years. He denied any trauma, fall or manipulation to the stump. His finger
stick was under control until the pain started, and remained above 300. On physical
exam he was afebrile, not in acute distress with blood pressure of 128/64, heart rate
74, respiratory rate 16, 97% saturation on room air. The stump had a narrow ulcer
of 2 cm in length without discharge. There was no erythema or fluctuation around the
lesion. Cleansing and sterile gauzes were applied. He was instructed not to wear the
prosthesis until further notice and to return to clinic in three days. Laboratory
findings at the first visit were within normal limits. On a subsequent visit, the
patient presented with a more severe pain and reported foul odor from the stump. The
exam showed a 2 cm sinus-tract formation extending medially to the left knee and the
gauze was blood-tinged. The odor was distinctive immideately after removal of the
gauze, which he changed with normal saline wet-to-dry dressing daily. The patient
was prescribed oral amoxicillin/clavulanic acid and evaluated by the surgery that
day, who performed debridement. After 10 days into treatment, he was evaluated again,
when he reported no foul odor but the same severe pain at the stump. Bone scan was
obtained and the result came back positive for osteomyelitis in the remaining tibia
extending to the proximal femor. The patient was admitted to the surgery service for
above knee amputation.
DISCUSSION
The regular stump examination is not recommeded as opposed to foot examination in
diabetic patients. Peripheral neuropathy is present in over 80% of diabetic patients
with foot lesions and the prevalence of diabetic foot ulcers has been estimated to
be 3–8%. However, the prevalence of ulcer formation after amputations is not known.
After diabetic patients undergo amputation, their risk of requiring a second amputation
increases dramatically. Fever, chills and leukocytosis are absent in two thirds of
patients with limb-threatening infection. Hyperglycemia is a common sign of limb-
or life-threatening infection. It is imperative that clinicians maintain a high index
of suspicion for serious infection when evaluating diabetic patients with sudden onset
of uncontrolled hyperglycemia. Clinicians should also pay a close attention to the
fitting of prosthesis and examine a stump regularly.
THIS RASH HURTS!
J. Willis
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117522)
LEARNING OBJECTIVES
1. Identify the clinical presentation of herpes zoster. 2. Recognize the presenting
symptoms that require intravenous therapy for herpes zoster.
CASE
A 26-year-old man presented with two days of a left-sided chest pain. The pain was
constant, sharp and radiated to the left arm. He was admitted to the hospital for
evaluation of acute coronary syndrome. His EKG's remained normal, as did serial troponin
I's. The intense pain in the left lower chest continued with radiation now to the
back. Two days following admission he noted an onset of the rash that he attributed
to a recent contact with shrubbery. Over the next day, it progressed to a weeping,
painful, pruritic rash along the left side of the lower chest. He noted associated
fever and chills. His examination was normal with the exception of a vesicular rash
along the T8 to T10 dermatome. There was exquisite tenderness to light touch along
this same area. There were no ocular abnormalities and his lung examination was normal.
His white cell count was 3,100 with 54% neutrophils; no bands. Remaining laboratory
studies were normal.
DISCUSSION
Herpes zoster is a vesicular reactivation rash from prior exposure to the varicella
virus. It frequently presents with a painful prodrome, with a subsequent vesicular
rash that follows within the next forty-eight hours. Knowledge of the prodrome is
important as other causes of chest pain can be falsely diagnosed. Excluding involvement
of the ophthalmic branch of cranial nerve V is also important, as intravenous acyclovir
is required to prevent corneal involvement and scarring. Hutchinson's sign is the
appearance of vesicles on the tip of the nose, suggesting cranial nerve V involvement.
The rash with herpes zoster is progressive, as if it is crawling along this skin (herpe-
(L); to crawl). The dermatomal pattern is diagnostic and distinguishes zoster from
other vesicular diseases (zoster- (G); girdle-like). The syndrome is also known as
shingles, from the Latin, cingella; meaning girdle-like.
TOO YOUNG FOR AORTIC DISSECTION?
J.L. Oyler
1. 1University of Chicago, Chicago, IL. (Tracking ID #115741)
LEARNING OBJECTIVES
1. Recognize and treat aortic dissection in atypical patients. 2. Diagnose bicuspid
aorta and manage complications like aortic dilation/dissection.
CASE
A 30 year old graduate student presents to Student Care with chest pain. One day prior
to admission he experienced chest pain while on the exercise bicycle at the gym. Chest
pain was sharp, substernal, non-radiating, 7/10, lasting seconds, pleuritic and associated
with SOB. He had 2 episodes of syncope lasting seconds, precipitated by N/V. He denied
diaphoresis, F/C/S, or viral symptoms. On physical exam he was afebrile, blood pressure
in each arm was 105/80, pulse 96, RA sat 98%. He was a pale appearing male in mild
distress due to pain. Cardiovascular exam revealed nl S1,S2 no M/G/R, lungs were clear,
abdomen was benign, pulses were equal. Initial labs revealed normal CBC, BMP, LFT's,
CK, MB, troponin, urine toxicology, RPR, ANCA, ANA and ESR. Initial EKG showed diffuse
ST segment elevation, PR elevation in AVR. CXR showed widened mediastinum. CT scan
showed pericardial effusion, anuerysmal dilation of the aortic root. Final diagnosis
was made by TEE revealing bicuspid aortic valve, aortic dissection 3 cm above aortic
root. He was taken to surgery, but had crushing substernal chest pain in the pre-op
area and died of pericardial tamponade.
DISCUSSION
Aortic dissection is an unlikely diagnosis in a young normotensive male presenting
with chest pain. In young patients predisposing factors for aortic dissection include:
vasculitis, collagen vascular disease, bicsupid aortic valve, aortic coarctation,
crack cocaine, and trauma. Although most aortic dissections present as chest pain,
when associated with syncope most patients have Daily type A dissections involving
the ascending aorta and increased incidence of tamponade and worse outcomes. CT, MRI,
and TEE are all recognized as effective imaging techniques for aortic dissection.
Treatment of aortic dissection includes blood pressure control and surgery to excise
the intimal tear. Bicuspid aortic valve (BAV) is the most common congenital cardiac
malformation. Males are affected 4:1. BAV is primarily diagnosed by echocardiography
after presence of aortic ejection click +/− systolic ejection murmur is detected.
Serious complications of valvular stenosis, regurgitation, infective endocarditis,
and aortic dilation and dissection occur in >33% of patients with BAV. Accelerated
degeneration of the aortic media, not valvular dysfunction, causes the vascular complications
of BAV. Even when BAV is replaced by prosthesis, abnormalities in aortic media can
cause aortic dilation. Antibiotic prophylaxis and blood pressure control are mainstays
of therapy. Patients with BAV should be monitored by echocardiography at regular intervals.
Once AI/AS, dilated aorta >4cm, increased LV size or decreased LV function occurs
BAV patients should undergo surgery.
TRANSIENT LEFT VENTRICULAR APICAL BALLOONING: A NOVEL HEART SYNDROME
H.L. Korlakunta
1; S.K. Thambidorai1; S. Denney1; I. Khan1. 1Creighton University, Omaha, NE. (Tracking
ID #115715)
LEARNING OBJECTIVES
To report a case of transient left ventricular apical ballooning without coronary
artery stenosis, which mimics acute myocardial infarction with electrocardiographic
changes and elevation of cardiac enzymes disproportionate to the extent of akinesia
of left ventricle.
CASE
A 43-year-old white female with past medical history of hypertension and hyperlipidemia
and family history of coronary artery disease presented with typical angina which
occurred while she was giving a briefing to a large group. Her medications included
hydrochlorothiazide and simvastatin. She was treated with sublingual nitroglycerin,
morphine, and a beta-blocker with resolution of symptoms. Physical examination was
unremarkable. Electrocardiogram showed minor non specific ST-T wasve changes and was
negative for myocardial ischemia and injury. Laboratory workup revealed elevated serum
cardiac troponin I (peak 2.03 ng/ml). Serum electrolytes were within normal limits.
Coronary angiogram was performed which showed normal coronary arteries; severe distal
anterior, apical, and distal basal hypokinesis; and ejection fraction of 25%. Echocardiogram
reveled low ejection fraction, hypokinesis of apical segments of anterior, inferior,
and lateral walls and distal segment of intraventricular septum. Repeat echocardiogram
3 days later showed improvement in regional wall motion abnormalities and ejection
fraction rose to 45%. Subsequent electrocardiograms showed diffuse T-wave inversion
with prolongation of QTc interval to 500 msec, which slowly reverted toward normal.
The QTc interval before discharge was to 452 msec. The patient was discharged on a
beta-blocker agent and an angiotensin-converting enzyme inhibitor and follow-up echocardiogram
in 4 weeks was recommended.
DISCUSSION
A novel cardiac syndrome of left ventricular apical ballooning was recently described,
which involves an acute onset of reversible left ventricular apical wall motion abnormalities
with chest symptoms, electrocardiographic changes and minimal elevation of cardiac
enzymes mimicking acute myocardial infarction. Patients have no angiographic evidence
of coronary artery stenosis. There is complete recovery of left ventricular function
in weeks. The precise etiologic basis of this syndrome is yet to be determined. Previous
studies have indicated that triggering factors such as emotional exposure and physical
stress may play a role in the pathophysiologic basis of this condition. This syndrome
might be one of the clinical models of stress-related sudden death. Awareness of this
syndrome is important because it mimics acute myocardial infarction and may inadvertently
expose patients to futile administration of thrombolytic agents. In addition, since
recurrence seems possible, there is need for prompt recognition and optimal treatment
of this novel heart syndrome.
TRANSVERSE MYELITIS SECONDARY TO HERPES ZOSTER IN AN IMMUNOCOMPROMISED PATIENT
A. Halat
1; J.T. Bates1. 1Baylor College of Medicine, Houston, TX. (Tracking ID #116903)
LEARNING OBJECTIVES
1. Recognize the clinical presentation of transverse myelitis. 2. Review the appropriate
evaluation and treatment of transverse myelitis.
CASE
A 70 year-old man with myasthenia gravis, status post thymectomy and now treated with
azathioprine and prednisone, presented with progressive leg weakness, bowel and bladder
incontinence, and skin lesions. He described a two week history of burning pain down
the back of his legs. Examination revealed vesicles on the roof of his mouth and on
his lower extremities. He had decreased strength and sensation in his bilateral lower
extremities, absent reflexes at the knees and ankles bilaterally, and a positive Babinski
sign on the right. Neither magnetic resonance (MR) nor computerized tomography (CT)
imaging could be obtained. A lumbar puncture was performed, and the CSF revealed a
negative gram stain, 112 white blood cells with 56% lymphocytes, an elevated protein
of 120, and a glucose 48% of the serum value. Given the strong suspicion of a viral
etiology, CSF was sent for herpes simplex (HSV) and varicella zoster (VZV) analysis
by polymerase chain reaction (PCR). PCR demonstrated the presence of varicella zoster.
Given this finding and the patient's clinical picture, it was felt that the patient
had a transverse myelitis secondary to varicella zoster. The combination of the patient's
myasthenia gravis and his immunosuppressive regimen was felt to have predisposed him
to this infection.
DISCUSSION
Transverse myelitis involves progressive limb weakness with loss of tendon reflexes
and a sensory level that is typically sudden in onset. While infection can cause transverse
myelitis, it is not the only cause. Imaging with either MR or CT should first exclude
myelopathy from a structural cause, such as a herniated disk, vertebral fracture,
or malignancy. In the absence of structural causes, transverse myelitis can result
from multiple sclerosis, systemic diseases such as Sjogren's syndrome and systemic
lupus erythematous, post radiation changes, infarction of the spinal cord, and infection.
In the absence of structural lesions, lumbar puncture should be performed to assess
the degree of inflammation, document any infection, and to ascertain the presence
of oligoclonal bands to assess for multiple sclerosis. Treatment depends on the underlying
etiology, but if VZV or HSV is suspected, then empiric treatment with acyclovir should
be started immediately while definitive PCR testing is pending. Unfortunately, in
cases of transverse myelitis secondary to VZV the response to treatment is limited,
and most patients do not recover full neurological function.
TROPICAL SPLENOMEGALY
S.C. Reddymasu
1; S. Alla1; S. Schlanger1. 1Creighton University, Omaha, NE. (Tracking ID #116488)
LEARNING OBJECTIVES
1. Describe the common causes of massive splenomegaly in the sub-Saharan African immigrant
population 2. Outline a cost-effective clinical approach to massive splenomegaly.
3. Discuss the treatment of tropical splenomegaly
CASE
A 35-year-old Sudanese woman who immigrated to the US 6 months ago reported abdominal
discomfort, fatigue, and low-grade intermittent fever, particularly at night. She
had no dyspepsia, hematemesis, melena, dysuria, or hematuria. She reported malaria
episodes, one requiring hospitalization in 1992, and a subsequent episode 7 months
ago. She denied kala azar, schistosomiasis, inborn errors of metabolism, and malignancy.
Family history for leukemias and lymphomas were negative. Examination was remarkable
only for massive splenomegaly. The platelets were 95,000. The hemoglobin was 12.8
gm/dl and she had 6,000 white cells with 8% eosinophils and 18% lymphocytes. Peripheral
smear was negative. Hepatitis B core antigen was present. Urinalysis and urine -human
chorionic gonadotropin were negative. Chest film was negative but computed tomographic
scan of the abdomen was remarkable only for splenomegaly measuring 16 cm. Urine and
stool examination for ova and parasites was negative. Total serum IgM was normal.
Treatment began with chloroquine for presumed tropical splenomegaly.
DISCUSSION
This 35-year-old Sudanese immigrant with recurrent malaria presented with massive
splenomegaly. Given her age and geographic background as well as the absence of evidence
of hematologic dyscrasias and malignancy, and inborn errors of metabolism, we considered
infectious etiologies. Schistosomiasis and kala azar were unlikely based on her clinical
picture. Her presentation was consistent with chronic malaria syndrome—caused by low-grade
antigenemia—or tropical splenomegaly—or hyperreactive malaria syndrome (HMS), thought
to be caused by hyperfunctioning B-lymphocytes in response to malaria antigen present
in blood. There is no clear-cut difference in diagnostic criteria or treatment between
these entities. In malaria-endemic regions, they are the leading cause of splenomegaly
(followed by lymphoma) when other entities cannot be demonstrated. Common tests done
for HMS are serum IgM and antimalarial antibodies, but they are insensitive, nonspecific,
and costly. Malaria parasite cannot be found on peripheral smear in HMS, though peripheral
smear done every 8 hours over 3 days can show malaria parasite in chronic malaria;
this is expensive and impractical. Consequently, a trial of weekly chloroquine for
6 months, checking for splenic regression (40%)—which is neither costly nor toxic,
is advocated for massive splenomegaly in malaria-endemic areas with no obvious cause.
Persistence of splenomegaly justifies evaluation for splenic lymphoma as well as assay
for anti-leishmanial antibodies. Tropical splenomegaly should be treated given its
association with splenic lymphoma and risk of hypersplenism.
TUBERCULOSIS LYMPHADENITIS: A PERSISTENT PAIN IN THE NECK
M. Lee
1; A. Kosmin1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116178)
LEARNING OBJECTIVES
1. Recognize that tuberculosis (TB) lymphadenitis is still very common worldwide.
2. Recognize diagnostic and treatment strategies for TB lymphadenitis.
CASE
A 32 year old West African female with AIDS (CD4 334) and previously treated latent
TB presented with four month history of progressive weakness and intermittent fevers/chills.
She also complained of headaches, anorexia and weight loss, night sweats, nonproductive
cough, nausea and post-tussive vomiting. One year prior, she had visited West Africa
for several months. On exam, she had rectal temperature 103.5 and pulse 111. She had
a 5 × 5 cm nontender mass at the anterior superior aspect of her right neck. She had
3/6 systolic murmur at the left apex. Lab data revealed WBC 5.4, Hgb 10.1, platelets
148. CXR, CT head and lumbar puncture were normal. Urine and blood cultures for bacteria,
fungi and mycobacteria, serologies for Bartonella hensalae, and malarial smears were
all negative. Echocardiogram and CT abdomen/pelvis were normal. Fine needle aspiration
of the right neck mass revealed lymphoid cells (some atypical), epitheloid cells,
RBCs and necrosis. Smears were negative for organisms, fungal elements and acid fast
bacilli. After much debate, the patient was discharged on a four drug regimen for
a presumptive diagnosis of TB lymphadenitis. Five weeks later, she was readmitted
for enlarging painful neck mass. Surgical exploration revealed an underlying cervical
abscess and lymph nodes that were positive for acid fast bacilli staining. Pathology
showed granulomatous lymphadenitis with areas of necrosis. Cultures grew pan sensitive
M. tuberculosis.
DISCUSSION
Tuberculous lymphadenitis remains a common cause of extrapulmonary TB worldwide. In
developing nations, it causes 43% of peripheral lymphadenopathy. In the United States,
5.4% of TB is extrapulmonary, and 31% of these cases are lymphatic. Clinical presentation
depends on the site of nodal involvement and the immune status of the patient. Immunocompetent
patients present with an isolated chronic, nontender lymphadenopathy. Immunocompromised
patients present with systemic symptoms and disseminated disease. The diagnosis of
TB lymphadenitis is suggested histologically with necrotizing or caseating granulomata
and confirmed with culture data from a lymph node biopsy. Fine needle aspiration (FNA)
is safe and inexpensive but has sensitivity of 60–70% due to sample error. Excisional
biopsy, the gold standard, is required if FNA is non-diagnostic. There is no consensus
as to whether surgical excision is sufficient to treat TB lymphadenitis. Therefore,
all patients are treated with a multidrug regimen, initiated prior to pathologic confirmation.
Treatment regimen and duration are similar to that of pulmonary TB. Relapse rates
of up to 3.5% have been reported.
WHY CAN'T MY PATIENT HEAR ME?
J.M. Weiss
1; J.M. Sosman1. 1University of Wisconsin Medical School, Madison, WI. (Tracking ID
#117050)
LEARNING OBJECTIVES
1. Identify extraintestinal manifestations of Ulcerative Colitis (UC). 2. Recognize
that there is a well-documented association between sensorineural hearing loss and
UC.
CASE
A 57 year-old man presented to his local MD with back pain. He was treated with Valdecoxib,
but subsequently developed oral ulcers thought to be secondary to this medication.
Two months later, he was found to have elevated liver function tests during a life
insurance evaluation. Initial workup with viral hepatitis serologies was negative.
Over the next three months, he developed a recurrent throbbing headache, jaw pain,
and vertigo with nausea and vomiting. He was admitted to his local hospital. His workup
included a normal head MRI/MRA but identified a microcytic anemia (Hct 35.5, MCV 80)
with guaiac positive stools, a WBC 11.1 K, a Plt 440 K, an ESR of 116, and a CRP of
5.3. His temporal artery biopsy was negative. He began to complain of left sided earache
and hearing loss and was transferred to our facility for evaluation. He was diagnosed
with an acute idiopathic sensorineural hearing loss. He also complained of eye “floaters”
and was found to have anterior iritis/uveitis. A liver biopsy to evaluate his abnormal
LFTs (ALKPhos 523, GGT 1231, AST 23, ALT 37) revealed a possible small duct sclerosing
cholangitis. Finally, his colonoscopy revealed quiescent colitis in the rectum, chronic
inflammation in the left colon, and evidence of previous ulceration in the right colon.
Although our patient never had problems with diarrhea—he was diagnosed with UC based
on the above constellation of extraintestinal manifestations.
DISCUSSION
Ulcerative Colitis is an inflammatory bowel disease (IBD) involving the mucosal layer
of the colon. UC is typically characterized by recurrent episodes of crampy abdominal
pain and diarrhea (often bloody), however, patients may present in a variable manner.
Extraintestinal manifestations are common and can occur in up to 25% of patients with
UC or Crohn's IBD. These manifestations include reactive arthropathy (up to 20%),
axial arthropathy, uveitis and episcleritis, skin lesions (erythema nodosum or pyoderma
gangrenosum), and primary sclerosing cholangitis (2-5%). Of these, uveitis, axial
arthropathy, and primary sclerosing cholangitis can occur at any time without active
colitis. Sensorineural hearing loss has a well-documented association with UC and
the relationship is speculated to be of autoimmune etiology (the prevalence of autoimmune
disorders occur in up to 10% of UC patients). Once recognized, immediate treatment
with steroids with or without immunosuppressive therapy is essential to prevent irreversible
hearing loss. Our patient was started on high-dose oral steroids, but unfortunately
continues to have significant hearing loss, tinnitus, and disequilibrium.
UNUSUAL NEUROLOGIC COMPLAINTS: CONSIDER MULTIPLE SCLEROSIS
N. Lischner
1. 1University of California, San Francisco, San Francisco, CA. (Tracking ID #116995)
LEARNING OBJECTIVES
1) Recognize signs and symptoms of multiple sclerosis. 2) Diagnose multiple sclerosis
using history, physical exam and central nervous system (CNS) imaging.
CASE
A 34 year old black male presented to his PCP with a six month history of progressively
worsening diplopia, clumsy gait, and episodes of extreme fatigue. A few weeks prior
to presentation, he also developed slurred speech. All symptoms seemed exacerbated
by hot baths and physical exertion. He denied pain, paresthesias, dysesthesias, bowel
or bladder changes, weakness, and vision loss. Remainder of ROS was negative. Physical
exam was notable for slightly slurred speech, normal visual acuity, left eye adductor
weakness, and bilateral hyperreflexic (3+) DTRs in the biceps, triceps, patella, and
Achille's tendon without clonus. Exam also revealed a subtle gait disturbance, bilateral
(L > R) dysmetria on finger-to-nose testing, and bilateral deficits on heel-to-shin
testing. The remainder of his exam was normal, including negative Lhermitte's and
Romberg signs. Brain MRI revealed extensive T2-weighted hyperintense lesions throughout
the corpus callosum, periventricular white matter, deep and subcortical white matter,
as well as left pons and middle cerebellar peduncle, consistent with a primary demyelinating
disease such as multiple sclerosis. No gadolinium enhancement to suggest an acute
demyelinating process was noted.
DISCUSSION
Multiple sclerosis (MS) is a chronic neurologic disease of autoimmune axonal demyelination.
Symptoms include fatigue, bowel, bladder, or sexual dysfunction, motor weakness or
spasticity, paresthesias, dysesthesias, ataxia, dysarthria, diplopia, vision loss,
gait disturbance, balance problems, vertigo, and pain. Uhtoff's phenomenon, which
is exacerbation of symptoms when the ambient body temperature is raised, is reported
by some patients. Signs include optic neuritis, ataxia, dysarthria, dysmetria, internuclear
ophthalmoplegia, clonus, dystonia, hyperreflexia, motor and sensory deficits. Lhermitte's
sign, which is the sensation of electric shock in the extremities when the neck is
flexed, can sometimes be elicited. Diagnosis of MS requires the presence of CNS lesions
separated in time and site. Imaging the CNS can rule out alternate etiologies, such
as infection or neoplasm. Brain or spine MRI is the usual modality. If MRI shows lesions
consistent with demyelination, it can support the clinical diagnosis of MS. T2-weighted
hyperdensities are typically found in the periventricular white matter, corpus callosum,
centrum semiovale, and less commonly in the deep white matter structures and basal
ganglia. Lesions are hypointense or not seen at all on T1-weighted imaging. Gadolinium
enhancement of lesions indicates active inflammation; enhancement usually remains
for 4–8 weeks after lesions become active.
URINARY URGENCY AS THE PREDOMINANT SYMPTOM OF NEURO. TB
S.G. Driscoll
1; D.T. Fisk1; M. Schapira1. 1Medical College of Wisconsin, Milwaukee, WI. (Tracking
ID #115957)
LEARNING OBJECTIVES
1. Recognize urinary urgency and intermittent back pain as indicators for CNS evaluation
in a patient with disseminated TB. 2. Diagnose neuro TB utilizing the most appropriate
radiological testing.
CASE
A 23 year-old, HIV negative man presented to the ER complaining of back pain and neck
mass. The pain and neck mass began one month prior and were accompanied by malaise,
drenching night sweats and an 8-pound weight loss. History was notable for immigration
from Mexico two years prior. After admission, fluid aspiration from the neck mass
grew pan-sensitive mycobacterium tuberculosis. Chest radiograph demonstrated a subtle
right middle lobe infiltrate; chest CT showed a cavitary lesion within the area of
the infiltrate. CT did not reveal any spine or bone pathology. The patient was initiated
on isoniazid, rifampin, pyrazinamide, pyridoxine, and ethambutol. For the next six
weeks, clinic visits documented complaints of urinary urgency that had predated anti-tubercular
medication initiation. Post-void residual was 30 cc; prostate exam and multiple neurological
exams were normal. Thoracic CT with contrast again detected no spine or cord pathology.
MRI with gadolinium, however, noted enhancing basal cistern meningies, cortical lesions
suggestive of tuberculomas, and evidence of epidural phlegmon and abscesses along
the entire length of the thoracic cord to L1, deforming and displacing the cord, highly
suggestive of neuro-TB. Steroids were initiated, isoniazid was doubled from 300 to
600 mg daily, ethambutol was discontinued, and his symptoms resolved within 2 weeks
of therapy. At no time did the patient have altered mental status, seizures, or focal
weakness.
DISCUSSION
This case demonstrates the importance of maintaining a high level of suspicion for
neuro-TB in a patient with known or suspected TB. Suspicion for CNS involvement should
be prompted not only by classically described neuro-TB symptoms of meningismis, mental
status changes and focal neurological deficits, but also by mild symptoms such as
urinary urgency and back pain. Urinary urgency has not been described as a presenting
symptom of neuro-TB, though urinary retention is a recognized complication of spinal
cord TB and meningeal TB has been implicated in diabetes insipidus development. These
symptoms should be recognized as indicators of potential CNS pathology and appropriate
diagnostic maneuvers pursued. This case was also remarkable in that MRI demonstrated
extensive disease in the face of very mild symptomatology, confirming the great sensitivity
of MRI for detecting neuro-TB. Given the growth of immunocompromised and immigrant
populations in the United States, and the global ascent of multi-drug resistant tuberculosis,
detection and treatment of TB will be an increasingly important aspect of health care
in the future.
VENTRICULAR SEPTAL RUPTURE IN A PATIENT WITH COCAINE ABUSE
K. Dyehouse
1; V.T. Martin1. 1University of Cincinnati, Cincinnati, OH. (Tracking ID #115676)
LEARNING OBJECTIVES
Recognize the clinic manifestations, diagnostic workup and management of ventricular
septal rupture.
CASE
A 51-year-old male, with untreated diabetes and hypertension presented with dyspnea
on exertion and lower extremity edema of two weeks duration. He also complained of
PND and orthopnea. There was no history of chest discomfort, diaphoresis, nausea,
vomiting, fever, chills or sweats. Physical exam revealed tachycardia, with a IV/VI
systolic murmur best heard at left lower sternal border with radiation to the right
sternal border, jugular venous distention, bilateral crackles to mid-lung fields,
and pitting edema to the mid-thighs. Laboratory data included negative cardiac enzymes
and troponin-T. The urine was negative for protein but positive for cocaine. Chest
x-ray revealed cardiomegaly and pulmonary edema. His electrocardiogram demonstrated
sinus tachycardia with right axis deviation, right bundle branch block and right ventricular
hypertrophy. An echocardiogram showed a large pericardial effusion, a ventral septal
defect and an apical ventricular aneurysm. Right heart catheterization revealed elevated
pressures (81/13 mmHg) and an oxygen saturation step-up from 53% to 71% in the right
ventricle. Coronary angiography revealed a total occlusion of the first marginal and
right coronary arteries. These findings were presumed to be a result of a myocardial
infarction possibly precipitated by recent cocaine abuse. The patient underwent a
patch closure of his ventral septal defect and is doing well in follow-up.
DISCUSSION
Acute coronary syndrome is the most common cardiac pathology associated with cocaine
abuse. Cocaine is attributable to approximately 25% of nonfatal myocardial infarction
in adults ages 18–48. Ventricular septal rupture is a rare complication of myocardial
infarction with an incidence of 1–3 percent. The incidence has decreased 10-fold with
the advent of thrombolytics. Clinical manifestations include: chest pain, shortness
of breath, hypotension, development of a holosystolic murmur at the lower left sternal
border with a thrill. Rapid diagnosis is essential to optimize survival. Doppler echocardiogram
is the diagnostic test of choice. Left ventriculography can also be diagnostic. Right
heart catheterization is useful in differentiating between papillary muscle rupture
and septal rupture. Treatment usually requires surgical intervention. Current guidelines
of the American College of Cardiology—American Heart Association recommend immediate
operative intervention on patients with septal rupture, regardless of their clinical
status. Medical therapies in the interim consist of mechanical support with an intra-aortic
balloon pump, afterload reduction, diuretics, inotropic agents and vasopressors. The
mortality rate is extremely high. The 30-day survival rate is 47 percent versus 24
percent in surgically versus medically treated patients.
VERTIGO: THE IMPORTANCE OF THE PHYSICAL EXAMINATION. A CASE REPORT AND REVIEW OF THE
LITERATURE
F.S. Drescher
1; D. Berz1; R. Weiss1; K. Mark1. 1Norwalk Hospital, Norwalk, CT. (Tracking ID #117401)
LEARNING OBJECTIVES
Dizziness and vertigo are common symptoms of patients presenting to general internists.
Though there are numerous etiologies for this complaint, a good history and physical
can often identify the diagnosis.
CASE
63 yo white male with 3 days of right-sided earache and vertigo. He had a history
of recurrent otitis media, hearing loss and bilateral mastoidectomy as well as diabetes.
Initial otoscopic examination suggested severe right-sided otitis externa and a perforation
of the right tympanic membrane. No nystagmus was noted. An ENT specialist was consulted
and treatment with topical eardrops, intravenous quinolones and oral meclizine was
begun. The patient's earache improved slightly but his vertigo persisted. On reevaluation
the patient noted that his symptoms were worsened when pressure was applied to his
right external meatus, which also resulted in the development of a profound nystagmus
(see photographs). When this finding was described to the ENT consultant, he felt
that it represented “the fistula sign” and recommended a CT-scan. This showed destruction
of the horizontal semi-circular canal and a discontinuity of the petrous apex and
tegmen tympani (figure 2). He was referred for surgical repair.
DISCUSSION
This case demonstrates the importance of the history and physical examination in the
evaluation of vertigo. Though there are several useful clinical signs for evaluation
of the vertiginous patient, in this case the fistula sign was most helpful. This test
is useful in examining patients with recurrent vertigo. A finger is applied to the
external meatus, which causes a pulse of air-transmitted pressure. If nystagmus is
induced in association with symptoms of vertigo, bony destruction of the inner ear
is likely. Demonstration of this sign as illustrated in this case, can result in prompt
radiologic evaluation and surgical referral
WAITING FOR THE T IDE TO TURN: EISENMENGER's SYNDROME
D. Garrett
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117463)
LEARNING OBJECTIVES
1) Recognize the signs and symptoms of secondary polycythemia. 2) Recognize potential
causes of secondary polycythemia. 3) Review effective treatment modalities for secondary
polycythemia.
CASE
A 33 year-old man presented with four days of headache, dizziness, and shortness of
breath. He had a history of double outlet right ventricle and surgical banding of
the right pulmonary artery at six months of age. His exam was remarkable for a ruddy
complexion, and a puritic rash on his head, neck, and trunk. His conjunctiva was bright
red and he had cyanosis around the lips and ears. A holosystolic murmur was heard
at the apex that decreased with hand-grip. His PMI was medially displaced. He had
clubbing and cyanosis in all extremities. The hemoglobin was 19; the platelet count
was 113; and the MCV was 77. By EKG, he had right-axis deviation with left and right
ventricular enlargement. An Echo demonstrated a significant VSD.
DISCUSSION
As corrective surgery for congenital heart disease has become more successful, more
and more patients are living long enough to present to the general internist's clinic.
Physicians should recognize the signs of residual congenital heart disease, and know
the appropriate time for referral and how to manage expected complications. This patient
illustrates the importance of the physician's vigilance in detecting the reversal
of flow through a VSD (Eisenmenger's syndrome). The signs of cyanosis and erythrocytosis
suggest the beginning Eisenmenger's syndrome that results as the right ventricle increases
in size and mass due to chronic pressure overload from the left-to-right VSD. The
intracardiac shunt leads to hypoxia and a resultant secondary polycythemia. Unlike
primary polycythemia, this is not associated with splenomegaly and thrombocytosis.
Although both disorders have an increased RBC mass, mast cell proliferation, plethora,
and microcytosis, patients with secondary polycythemia have suppressed erythropoietin
and thus have thrombocytopenia as opposed to a thrombocytosis. Treatment by phlebotomy
is based on symptoms, and an effort to maintain a hematocrit below 45. Iron supplementation
is indicated in patients with secondary forms of polycythemia, where as it is contraindicated
in primary forms. Oxygen supplementation for relief of hypoxia is a mainstay of treatment
plans for these patients.
WEGENER's GRANULOMATOSIS: DIAGNOSTIC CHALLENGES IN EARLY STAGES
H. Khurana
1; S. Chittivelu1; L. Cation1. 1University of Illinois at Peoria, Peoria, IL. (Tracking
ID #115856)
LEARNING OBJECTIVES
To recognize chronic sinusitis and rhinitis as the most common presenting symptoms
for Wegener's Granulomatosis (WG). To underscore that a strong index of suspicion
is required for early diagnosis. To recognize that simple tests like C-ANCA and nasopharyngeal
biopsy can help establish an early diagnosis of WG and that prompt treatment may potentially
arrest disease progression.
CASE
An 18 year old white male was transferred to our institution for evaluation and management
of acute renal failure and cavitating pulmonary nodules. He sought medical attention
for a one-week history of nausea, vomiting, anorexia, arthralgias and non-productive
cough. His medical history was significant for chronic rhinitis, sinusitis and repair
of perforated nasal septum. Examination revealed pallor and saddle nose deformity.
Laboratory abnormalities included mild leucocytosis, elevated BUN, creatinine and
ESR. Urine analysis showed hematuria and proteinuria. ANA and anti GBM was negative.
The diagnosis of Wegener's Granulomatosis (WG) was confirmed with a positive C-ANCA
and lung biopsy. Patient responded to cyclophosphamide and prednisone therapy.
DISCUSSION
WG is an uncommon disease with an estimated occurrence of 3/100,000 population. The
age of onset varies from 5 months to 60 years with a median of 40 years. Less than
15% of the cases occur below the age of 20. There is a female preponderance in younger
age groups, as compared to adults where the gender difference is not marked. The “limited
form” of WG is confined to upper and/or lower respiratory tract whereas the “classic
form” also involves the kidneys. Most patients seek medical attention for symptoms
like chronic sinusitis and rhinitis. However, there is progressive involvement of
the lungs and kidneys in majority of patients by the time the diagnosis is established.
Diagnosis in early stages of the disease can be established by keeping a high index
of suspicion and simple tests like C-ANCA and nasopharyngeal biopsy. Early diagnosis
and prompt use of appropriate therapy can slow down and may even arrest the progression
of this potentially fatal disease
WEIGHT LOSS IN A PATIENT WITH METASTATIC MELANOMA
E.A. Sastre
1; C. Bates1. 1Beth Israel Deaconess Medical Center, Boston, MA. (Tracking ID #116976)
LEARNING OBJECTIVES
1) Recognize that new symptoms in cancer survivors often indicate common benign conditions
as opposed to recurrent malignancy; 2) List manifestations of hyperthyroidism; 3)
Define the association between hyperthyroidism and hypercalcemia.
CASE
A 53 year-old female with history of hypertension and metastatic melanoma presents
with weight loss and diarrhea. She was diagnosed with melanoma which metastasized
to the lungs and liver in 1994. Treatment with interleukin 2 resulted in complete
remission. On presentation, she described a 13 pound weight loss, abdominal cramping,
non-bloody diarrhea, and fatigue. She denied fevers or chills but noted pruritus.
Physical examination was notable for a normal thyroid gland, a normal cardiopulmonary
exam, and mild left lower quadrant abdominal tenderness. Concern for recurrent melanoma
was raised; her metoprolol was discontinued as she thought it was causing fatigue.
The patient subsequently developed palpitations and dizziness. Laboratory values revealed
normal liver function tests, calcium 10.9, an undetectable TSH, T4 of 5.2, and a T3
of 622. The patient restarted metoprolol; symptoms improved. Thyroid uptake scan demonstrated
diffuse increased uptake consistent with Grave's disease. She was started on methimazole.
Parathyroid hormone level was suppressed and both PTH-related protein and Vitamin
D levels were normal. Treatment normalized thyroid function tests and serum calcium.
DISCUSSION
Physicians may assume that new symptoms in patients with prior malignancy are secondary
to disease recurrence. However, these patients are also susceptible to common benign
illnesses, such as hyperthyroidism, which may present similarly to metastatic disease.
Symptoms include weight loss, diarrhea, palpitations, anxiety, and heat intolerance.
Physical examination may reveal tachycardia, fever, moist skin, thyroid gland irregularities,
arrythmias, and brisk reflexes. Suppressed TSH and elevated levels of T4 and T3 confirm
the clinical suspicion. The most common etiology of hyperthyroidism, Grave's disease,
is an autoimmune disorder; antibodies directed against TSH receptors on the thyroid
gland increase thyroid hormone synthesis and gland size. The patient classically presents
with diffuse goiter (90%), ophthalmopathy (50%), and dermopathy (<5%). Grave's disease
is diagnosed by diffuse increased uptake on radioactive iodide uptake scan. One of
the less common manifestations of hyperthyroidism is hypercalcemia. Thyroid hormone
stimulates bone resorption through action on T3 receptors on osteoblasts and osteoclasts.
In addition, elevated IL-6 levels also stimulate osteoclast activity. Approximately
8% of patients with clinical hyperthryroidism have hypercalcemia that usually resolves
with treatment of hyperthyroidism.
WERNICKE ENCEPHALOPATHY
F. Nahab
1; H. Limkemann2. 1University of California, Los Angeles, San Fernando Valley Program,
Sylmar, CA; 2University of California, Los Angeles, Sylmar, CA. (Tracking ID #102043)
LEARNING OBJECTIVES
1. Recognize the clinical manifestations of Wernicke Encephalopathy (WE). 2. Consider
WE as part of the differential diagnosis of any acute oculomotor dysfunction and/or
ataxia. 3. Review the CT and MRI findings in WE.
CASE
A 53 year old Hispanic-German gentleman with a history of hypertension and hyperlipidemia
presented with double vision and inability to walk without assistance for 12 hours.
He denied any witnessed ALOC, headache, weakness, trauma or fever. The patient admitted
to a 20+ year history of binge drinking with his last binge occurring 3 weeks prior.
Family history was significant only for a grandfather who died of a stroke. On exam,
the patient's blood pressure was 143/95. Although alert and oriented × 4, on neurological
examination the patient was found to have an isolated right medial rectus palsy and
an inability to converge. Otherwise, cranial nerves were intact. Strength was 5/5
in BUE and BLE with 2+ DTRs symmetrically. Vibratory sensation was intact. No ataxic
extremities were noted. Patient's gait was broad-based but steady however on tandem
gait marked ataxia was noted. Romberg test was negative. Hemoglobin was 13.1 with
an MCV of 101.3. Total cholesterol was 239 and triglycerides were 613. Liver function
tests and chemistry panel were normal. The differential diagnosis included stroke,
WE and multiple sclerosis so the patient was started on IV hydration supplemented
with thiamine, folate, and MVI while awaiting imaging. CT showed no evidence of mass,
bleed, or midline shift. MRI findings on T1- and T2-weighted, FLAIR, and DW images
showed no evidence of acute stroke but noted increased signal intensity of the mamillary
bodies in the T2-weighted image and showed mild periventricular white matter changes
consistent with microvascular ischemic disease. Within 12 hours of the patient's initial
presentation, the medial rectus palsy had resolved, convergence was intact, ataxia
on tandem gait had resolved, and patient was discharged home.
DISCUSSION
WE is a neurological syndrome that can include oculomotor dysfunction, ataxia and/or
disturbances of consciousness that range from mild confusion to coma. Clinical features
develop over a few days to weeks and result from a lack of thiamine. Neuroradiology
findings on CT scan include hemorrhages of the mamillary bodies. MRI findings are
best visualized on T2-weighted images and include hyperintensity of the mamillary
bodies, periaqueductal area, hypothalamus, thalamus, cerebellum and/or cerebral cortex.
Often, administration of high doses of thiamine may resolve symptoms though untreated
WE may be fatal or result in permanent neurologic damage. Therefore, it is important
to consider WE in the differential diagnosis of any acute oculomotor dysfunction,
ALOC and/or ataxia so as to begin thiamine supplementation early.
WERNICKE's ENCEPHALOPATHY FOLLOWING 5-FLUROURACIL THERAPY IN A WOMAN WITH BREAST CANCER
A. Byrnes
1; L. Coberly1. 1University of Cincinnati, Cincinnati, OH. (Tracking ID #115619)
LEARNING OBJECTIVES
1. Diagnose and act quickly to reverse Wernicke's Encephalopathy. 2. Recognize the
potential side effects of chemotherapeutic agents such as 5-Fluorouracil and treat
prophylactically or monitor closely to minimize complications.
CASE
Medication side effects are often overlooked when patients develop serious illness.
We present an unusual case of a complicated Wernicke's Encephalopathy (WE) in a patient
with recent neoadjuvant chemotherapy including 5-fluorouracil (5-FU). A 52-year-old
female with a history of infiltrating ductal carcinoma (T2NXM0) presented with altered
mental status. Her physical exam was normal except for a decreased level of alertness
and poor cooperation. She had recently been admitted with similar symptoms, and had
a normal head CT, a normal brain MRI with gadolinium, and three lumbar punctures,
which were negative (including cytology) except for elevated protein. EEG showed moderate
generalized slowing with epileptiform discharges.
DISCUSSION
This admission, repeat EEG on dilantin therapy revealed moderate to severe generalized
slowing without epileptiform discharges. Head CT was normal. Suddenly, the patient
became apneic (3 breaths/minute) and required intubation. She had a repeat MRI, revealing
enhancement of the mamillary bodies consistent with WE, changes which, in retrospect,
were present to a lesser degree on her prior MRI. She was started on intravenous thiamine
and had an immediate increase in her spontaneous respiratory rate to normal. Her cognitive
function returned. Unfortunately, she developed line sepsis with bacteremia and fungemia
leading to a critical illness polyneuropathy, which prevented extubation. WE, caused
by thiamine deficiency, is characterized by a constellation of neurologic abnormalities
including a global confusional state, disorientation and lethargy, nystagmus and truncal
ataxia. Respiratory depression is uncommon. Typically, EEG reveals diffuse slowing,
and CSF is normal except for elevated protein, as in our patient. 5-FU therapy increases
the risk of thiamine deficiency, and hence WE, as it blocks the conversion of thiamine
to its active metabolite thiamine pyrophosphate (TPP). Patients with deficiency of
dihydropyrimidine dehydrogenase (DPD, the enzyme responsible for 5-FU metabolism)
are at greater risk of this adverse effect of 5-FU. Cancer patients appear to be at
increased risk for thiamine deficiency. At baseline, they tend to have low levels
of thiamine, as evidenced by liver biopsy and bioassay. Cancer patients also appear
to have an increased incidence (3%) of DPD deficiency, thus making them more susceptible
to the adverse effects of 5-FU. Careful monitoring, and prophylactic thiamine administration
can be helpful.
WHAT CAUSED THE FEVER?
A. Agha
1; A. Kolpakchi1. 1Baylor College of Medicine, Houston, TX. (Tracking ID #117405)
LEARNING OBJECTIVES
1. Review the definition of fever of unknown origin (FUO). 2. Construct a differential
diagnosis of FUO. 3. Recognize that chronic bursitis can cause prolonged fever and
constitutional symptoms.
CASE
A 68 year-old male without significant past medical history presented to his primary
care provider with a one month history of malaise, anorexia, and 5lb weight loss.
He was noted to have temperature up to 101 F on three consecutive clinic visits. The
initial outpatient work up including CBC, liver function tests, chest x ray, urine
and blood cultures were negative. He was then admitted to the hospital for work up
of fever of unknown origin. Vital signs revealed temperature of 101 F, BP 140/80,
RR 14, HR 80. Physical exam was unremarkable except for a thickened, non-tender left
prepatellar bursa with small effusion. No warmth or erythema was noted. His labs revealed
elevated sedimentation rate at 39 (0-20) and elevated C-reactive protein at 12 (normal
being negative). The rest of the work up including RPR, MHA-TP, HIV, rheumatoid factor,
ANA, hepatitis panel, urine and blood cultures, CXR, CT scan of thorax and abdomen
was all negative. The patient continued to have elevated temperature up to 101.5 F
while in the hospital, and the etiology of his fever remained unknown. The patient
was reexamined again, and the previous non-tender thickened left prepatellar bursa
was aspirated with 9cc of thick amber fluid obtained. Culture was positive for Staphylococcus
Aureus sensitive to nafcillin. The patient was treated with nafcillin for 4 weeks
with complete resolution of his symptoms.
DISCUSSION
The fever of unknown origin (FUO) is defined as fever >38.3 C on several occasions,
duration of fever >3 weeks, and uncertain diagnosis after one week of study in the
hospital. Differential diagnosis of FUO includes four major categories: infections
(30%), malignancies (30%), collagen vascular diseases (10%) and unknown (30%). Interestingly,
the FUO in our patient was from his chronic prepatellar bursitis. Literature search
failed to produce a single case report of septic bursitis as the etiology of FUO.
Prepatellar bursa is one of few bursas that can become infected, most commonly by
Staphylococcus Aureus and other gram-positive organism (80%). Prepatellar bursitis
becomes chronic in approximately 5% of patients. Bursa aspiration is indicated for
diagnostic and therapeutic purposes. The treatment is with use of antibiotic for duration
of 4 weeks.
WHEN A ROSE IS JUST A ROSE: A CASE OF MONONUCLEOSIS
R. Ashkenazy
1; C. Bates1. 1Beth Israel Deaconess Medical Center, Boston, MA. (Tracking ID #116819)
LEARNING OBJECTIVES
1) Diagnose infectious mononucleosis (IM) using clinical and laboratory parameters;
2) Recognize the limitations of laboratory testing for IM.
CASE
A 37-year-old man had progressive fatigue and 5 days of temperature to 102.5 F, without
sore throat. Physical exam was notable for temperature of 100 F, pulse of 100, pharyngeal
erythema and cervical lymphadenopathy. The liver and spleen were not enlarged. WBC
was 2.2 K with 28% PMN, 9% bands, 37% lymphs and 18% atypical lymphocytes. The platelet
count was 97 K. Liver tests were elevated with AST of 373, ALT of 542 and alkaline
phosphatase of 304. Monospot test was negative. EBV VCA-IgM, EBV VCA-IgG and EBV EBNA-IgG
antibodies were negative. A throat culture was positive for rare group A strep; penicillin
was prescribed. A rash developed 6 days later. Because the patient's wife was pregnant,
the patient and his wife were tested for toxoplasmosis and CMV; serologies were negative.
Over the next 8 days, he had increased anorexia and somnolence. Tests for HIV and
hepatitis A, B and C were negative. He was referred to an infectious disease specialist.
Four days later he was admitted for his deteriorating clinical state and uncertain
diagnosis. Repeat monospot test and EBV VCA-IgM antibody were positive; EBV VCA-IgG
and EBV EBNA-IgG antibodies were negative. Management was supportive and he recovered.
DISCUSSION
IM is most often caused by Epstein-Barr virus (EBV), a herpesvirus spread in saliva
and, less commonly, through intimate contact or blood transfusions. Fever, pharyngitis
and lymphadenopathy, with transient heterophile antibodies and atypical lymphocytes,
characterize classic IM. Additional findings include fatigue, splenomegaly, rash (common
after administration of ampicillin/amoxicillin) and various neurologic syndromes.
EBV induces heterophile antibodies within 1 week of symptoms. The monospot test, a
latex agglutination assay, and enzyme-linked immunosorbent assay (ELISA) are rapid
diagnostic tests against these antibodies with a sensitivity of 86% and specificity
of 100%. Heterophile antibodies peak between the second and fifth week of infection
and may persist for up to a year. Epstein-Barr viral capsid antigen (VCA) antibodies
are also diagnostic. IgM antibodies are expected at presentation, persist for 1 to
2 months and mark acute infection. IgG antibodies are expected at presentation, persist
for life and mark acute or prior infection. IgG antibodies to EBV nuclear antigen
(EBNA) appear 6 to 12 weeks after symptom onset and persist throughout life; they
exclude acute infection early in an illness. This patient's initially negative EBV
VCA IgM and IgG and concerns about perinatal transmission led to testing for other
pathogens of mononuclosis-like syndromes. Of these, CMV is most common. Additional
testing in EBV-negative patients depends on risk factors for, and clinical implications
of, other infections.
WHEN ADDITIONAL HISTORY SOLVES THE MYSTERY: A CASE OF CAROTENOSIS CUTIS
M. Hadian
1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116190)
LEARNING OBJECTIVES
1. Recognize the clinical manifestations of beta-carotenemia. 2. Review the diagnostic
and therapeutic approach for beta-carotenemia. 3. Recognize the importance of returning
to the patient for additional history taking when diagnosis is unclear.
CASE
A 61year-old previously healthy Asian female presented complaining that she had become
yellow, especially during the prior week. She denied nausea, vomiting, diarrhea, constipation,
abdominal pain, anorexia, weight loss, fever, chills, change in urine/stool color.
She said she felt completely healthy, but she and her family were concerned about
her marked skin discoloration. She is a nurse, but denied needle sticks or hepatitis
exposures. Her only medication was a daily multi-vitamin. She denied alcohol, tobacco
or drug use. On exam, she had diffusely yellow skin with deeper discoloration of palms
and soles. She did not have scleral icterus. Her chemistries, CBC, TSH, cholesterol,
liver function tests were all normal. Her urine was microscopically normal, but had
an orange-yellow discoloration. At this point, further questioning revealed that the
patient was a vegetarian who ate a lot of green leafy vegetables and carrots. She
had recently increased her carrot intake to 2–3 pounds/day. Subsequently, serum carotene
was ordered. The patient had a value of 322 (normal 6–77 mg/dl). She was reassured
that this condition is benign and related to the consumption of too many carrots and
yellow vegetables. Patient was discharged with appropriate dietary instructions. One
week later she called to inform us that her skin color was back to her normal.
DISCUSSION
Carotenosis cutis, yellow-orange coloring of the skin, results from excessive intake
of vitamin A precursors in food, principally fruits and vegetables. Natural beta-carotene
acts as an anti-oxidant, but in excess amounts can lead to skin discoloration. The
palms and soles are predominantly involved. The yellowing of the skin is differentiated
from jaundice in that there is no scleral icterus. Other than cosmetic changes, carotenemia
is not harmful. Hypothyroidism and smoking makes patients particularly susceptible
to carotenemia. The omission of carrots and other colored fruits and vegetables from
the diet leads to the rapid disappearance of the hyperpigmentation. Our case illustrates
the importance of returning to the patient for additional history taking, especially
when the case does not follow a common, well-known pattern.
WHEN AMOXICILLIN ATTACKS: A CASE OF SERUM SICKNESS-LIKE REACTION
J.A. Chang
1; C.M. McEvoy2. 1Creighton University, Omaha, NE; 2University of Nebraska Medical
Center, Omaha, NE. (Tracking ID #115552)
LEARNING OBJECTIVES
1) Recognize the clinical manifestations of serum sickness 2) Diagnose serum sickness
accurately and quickly 3) Treat serum sickness in an appropriate fashion.
CASE
Our patient is a 29-year-old Caucasian male. 15 days before the initial visit to allergy
clinic, he had received Amoxicillin for sinusitis. 9 days later, he saw redness on
his palm and the sides of his fingers; followed by progressive swelling, erythema,
pruritis, and burning of his hands and feet, particularly on the dorsal surfaces.
White rings appeared on his hands the next morning. He had blotchy erythema on his
face, hands and feet. The hands and feet were swollen and very tender at the wrists
and ankles. He was given a loading dose of prednisone (80 mg) by his PCP, followed
with a Medrol Dose Pack for erythema multiforme. He initially improved, then developed
worsening symptoms. 3 days before the allergy visit, he required subcutaneous epinephrine
for acute angioedema. He presented to allergy clinic with severe, crippling joint
pain. His temperature was 38.1. Diffuse, blotchy edema with linear wheals and flares
over the face, trunk, and extremities was noted. ESR was 38, C3 was 161 and C4 was
33, with a CH50 of 199. Urinalysis showed protein of 30 mg/dL and blood of 25/uL.
He was diagnosed with serum sickness secondary to amoxicillin and started on high-dose
prednisone, which was slowly tapered over several weeks. His symptoms improved on
this regimen. He has been symptom-free since about 4 weeks after the onset of symptoms,
and has had no recurrences.
DISCUSSION
Serum sickness is the prototype of immune complex (type III) immune reactions. True
serum sickness is caused by the administration of heterologous sera; serum sickness-like
reaction results from non-protein drugs. Virtually all patients have fever and cutaneous
reactions. Joint pain with or without swelling is present in about 2/3 of patients.
Lymphadenopathy is also commonly seen. The pathophysiology is due to a combination
of antigen with IgG or IgM immune complex deposition, leading to the release of vasoactive
amines, which increase vascular permeability. The usual onset is 7–14 days after exposure
to a trigger (commonly beta-lactam antibiotics); however, in a previously sensitized
patient, symptoms may appear 2–4 days after exposure. The first line of treatment
is to stop the suspected inciting agent; antihistamines and NSAIDS are used for pruritis
and arthralgia. If severe symptoms exist, corticosteroids should be used for 10–14
days. Shorter courses are associated with recurrent symptoms which are more difficult
to control. Serum sickness is likely to recur on reexposure and may not be predicted
by skin testing.
WHEN GOOD INTENTIONS ARE NOT ENOUGH: A TRAGIC CASE OF I.V. PHENYTOIN USE
S. Daya
1; S. Jagadeesh1; N. Shaikh2. 1York Hospital, York, PA; 2Mercy Catholic Medical Center,
Lansdowne, PA. (Tracking ID #117245)
LEARNING OBJECTIVES
1) Recognize the increased risk for adverse events with phenytoin use in patients
with multiple comorbidities. 2) Recognize the importance of the appropriate rate of
parenteral phenytoin administration in high risk patients.
CASE
A 37 year old Afro-Caribbean male with a known history of End Stage Renal Disease
and liver cirrhosis presented to the renal unit to undergo his scheduled dialysis.
During dialysis he was noted to be diaphoretic and tachypneic, this was followed by
an episode of grand mal seizure which was treated with lorazepam given intravenously.
Patient had a second episode of grand mal seizure, given the recurrent nature phenytoin
was administered with a loading dose followed by continuous infusion. The seizure
episode was successfully terminated with the above treatment regime. Concurrent cardiac
monitoring during this period showed an initial sinus rhythm quickly change to sinus
bradycardia and second degree A-V block which deteriorated to asystole and cardiac
arrest. Phenytoin infusion was discontinued and cardiopulmonary resuscitation (CPR)
initiated. Patient was successfully resuscitated after 12 minutes with return to sinus
rhythm and stabilization of hemodynamic parameters. He needed endotracheal intubation
and mechanical ventilation for airway protection and oxygenation. Eventually the patient
was extubated and a neurological evaluation revealed the patient to have significant
cognitive and motor deficits which required long term nursing home care. An electroencephalographic
study showed neurological damage consistent with hypoxic encephalopathy.
DISCUSSION
Phenytoin is a commonly prescribed anticonvulsant used to treat most types of seizure
disorders and status epilepticus. Parenteral phenytoin administration is commonly
undertaken with careful cardiac monitoring due to the risk of cardiac arrhythmias.
The potential cardiac adverse effects include atrial and ventricular conduction disturbances,
hypotension, ventricular fibrillation and reduced cardiac output. Cardiac effects
are thought to be secondary to the propylene glycol diluent of the parenteral product.
Phenytoin in its parenteral form is dissolved in 40% propylene glycol and 10% ethanol.
Caution is advised with phenytoin use in any patient with cardiac disease because
adverse effects may be potentiated or exacerbated. The drug is absolutely contra-indicated
in patients with cardiac conduction abnormalities. Reactions to parenteral phenytoin
occur more often in the elderly or in patients with other significant comorbidities
such as renal or hepatic failure. The rate of intravenous administration of phenytoin
is critically important to avoid or limit adverse cardiovascular events that could
lead to serious long-term consequences.
WHEN TISSUE CAN'T ALWAYS BE THE ISSUE: USING CLINICAL CRITERIA TO DIAGNOSE CHRONIC
NECROTIZING PULMONARY ASPERGILLOSIS
R. Pechulis
1; B. Taqui1. 1Temple University, Philadelphia, PA. (Tracking ID #116208)
LEARNING OBJECTIVES
1. Recognize Chronic Necrotizing Pulmonary Aspergillosis (CNPA) as a clinical entity
distinct from other forms of Aspergillus pulmonary disease. 2. Recognize risk factors
and diagnostic criteria for CNPA.
CASE
A 55 year old African American female with sarcoidosis presented with one week of
fever, nightsweats, anorexia and cough productive of dark sputum. She had a similar
episode associated with hemoptysis 5 months prior. At that time, CXR revealed mycetoma
and sputum grew Aspergillus fumigatus. On exam, she had T = 101.6, RR = 25. She had
left apical bronchial breath sounds and right basilar rales. Lab data revealed Na
125, WBC 11. CXR showed a new thick walled left apical cavitary lesion with associated
pleural thickening, parynchemal consolidation, diffuse reticulo-nodular interstital
densities. The previously diagnosed right upper lobe mycetoma was not seen. The patient
was treated for community acquired pneumonia, but remained febrile and symptomatic.
Multiple sputum cultures grew Aspergilllus fumigatus and the patient had a bronchoscopy
with trans-bronchial biopsy. The bronchoalveolar lavage culture was positive for Aspergillus
fumigatus. Biospy specimen showed nonspecific inflammatory cells. After lengthy discussion
with our consultants and patient, open lung biopsy was deferred and the patient was
started on voraconazole for chronic necrotizing pulmonary aspergillosis. Since then,
she has had sustained clinical and radiologic improvement.
DISCUSSION
Chronic necrotizing pulmonary aspergillosis (CNPA), also known as semi-invasive or
chronic granulomatous aspergillosis, was first described in 1981. It is an indolant,
locally invasive aspergillus infection without vascular invasion or dissemination,
as seen in invasive pulmonary aspergillosis (IPA). It occurs in patients with underlying
lung disease or mild immunocompromised states. This population is compromised enough
to grow the fungus, but healthy enough to avoid the rapid demise that occurs in IPA
patients. CNPA patients present with cough and constitutional symptoms. Hemoptysis
occurs in 10% of cases. Radiologic studies reveal indolent upper lobe consolidation
associated with pleural thickening. Cavitation and/or mycetoma occur in 50% of cases.
The diagnosis is suggested by clinical presentation, isolation of Aspergillus from
pulmonary secretions, and exclusion of other etiologies (anaerobes, mycobacteria,
histoplasmosis, coccidiomycosis). The diagnosis is confirmed by pathologic evidence
of tissue invasion or a response to specific antimycotic drugs. Transbronchial and
percutaneous biopsies have low yield. Most patients have comorbid pulmonary conditions
that make an open lung biopsy a high risk. The duration of therapy is not well established.
WHEN URGENCY IS MORE THAN AN OVERACTIVE BLADDER: INTERSTITIAL CYSTITIS
C.L. Spagnoletti
1; M.A. McNeil1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #115016)
LEARNING OBJECTIVES
1) Recognize the clinical presentation of interstitial cystitis (IC) 2) Diagnose IC
in the patient with urinary urgency and frequency 3) Review the therapeutic options
for IC.
CASE
Ms. G. is a 50 year old female who presented with complaints of daily urinary frequency
and urgency and intermittent bladder pain for 8 weeks. She reported voiding up to
15 times per day, without an increase in her fluid intake or change in medications.
Her bladder pain improved with voiding. She denied dysuria, hematuria, recent UTI,
history of radiation therapy or abdominal or pelvic surgery. Her past medical history
was significant for chronic low back pain. Her medications included ibuprofen and
cyclobenzaprine. She was in a same-sex relationship and denied a history of sexual
abuse. On pelvic exam, she complained of bladder tenderness on palpation of the anterior
vaginal wall. Vaginal wet prep, KOH slide, GC and chlamydia were negative. Urinalysis,
culture, and cytology were unremarkable. Cystoscopy revealed a decreased bladder capacity
and presence of glomerulations after hydrodistention. A diagnosis of IC was made.
She was started on amytriptyline, hydroxyzine, and pentosan polysulfate sodium. Within
three months, she noted significant improvement in her symptoms of urgency, frequency,
and pain.
DISCUSSION
IC is a poorly understood condition affecting up to 700,000 people in the U.S. Most
are Caucasian and women make up 90%. The median age of onset is 43. It is a severe
and debilitating chronic pain syndrome that afflicts the bladder, and is characterized
by urinary urgency, frequency and bladder pain in the absence of other definable pathology.
Patients present with urinary urgency and frequency early in the disease and later
develop pain symptoms including suprapubic or pelvic pain, or dyspareunia. Dysuria
and incontinence are not typical. IC is also characterized by flare-ups and remissions.
There are many proposed theories of pathogenesis, but the cause is not known. The
differential diagnosis is broad and includes diseases which affect the urinary, gynecologic,
and gastrointestinal systems. IC is mainly a diagnosis of exclusion. 95% of IC patients
complain of a tender bladder base during pelvic exam, and the presence of this in
a patient with classic symptoms is highly suggestive. Urinalysis, culture, and cytology
are negative. An abnormal cystoscopy is diagnostic, but is only indicated in refractory
cases. If performed, reduced bladder volumes, glomerulations after hydrodistention,
and/or Hunner's ulcers are seen. Therapy is directed at symptom reduction and improved
quality of life. The mainstay of therapy includes the combination of tricyclic antidepressants,
antihistamines, and pentosan polysulfate sodium, plus dietary modification and pelvic
floor physical therapy. For more severe cases, intravesicular therapy or cystectomy
have been used.
WHERE's THE BEEF? AN INBORN ERROR OF METABOLISM PRESENTING AS A SEIZURE IN AN ADULT
S.D. Sisson1; D. Chaupin
1; E. Schmidt1. 1Johns Hopkins University, Baltimore, MD. (Tracking ID #117081)
LEARNING OBJECTIVES
1) Recognize a rare cause of seizure in an adult 2) Understand the clinical sequelae
of ornithine transcarbamylase deficiency.
CASE
A 53-year-old female with a history of achondroplasia was admitted for persistent
abdominal pain, nausea, vomiting, and diarrhea. Past medical history was notable for
chronic abdominal pain, attributed to chronic pancreatitis. In reviewing the patient's
history of abdominal pain, she reported having abdominal pain and headaches since
childhood, which she associated with eating meat. As a result, the patient had altered
her diet to consist primarily of grains. Physical examination was notable for a cachectic,
chronically ill-appearing female, lying still in bed. She had diffuse abdominal tenderness
with rebound but without guarding. A CT scan of the abdomen showed acute pancreatitis
with peripancreatic inflammation and multiple pseudocysts. As treatment, the patient
was started on a standard parenteral solution of electrolytes, trace elements, glucose,
amino acids, and lipids. Shortly after initiation of parenteral nutrition, the patient
became somnolent and seized, confirmed by EEG. Benzodiazepines and phenytoin were
administered, without a response. Parenteral nutrition was stopped, and on further
evaluation an ammonia level came back profoundly elevated (190 mcg/dl). Liver function
tests were normal. An elevated ammonia level in the setting of normal liver function
suggested the diagnosis of a urea cycle disorder. An allopurinol challenge test was
performed and confirmed the diagnosis of ornithine transcarbamylase deficiency.
DISCUSSION
In the normal host, ammonia produced by protein catabolism is converted to urea by
a series of steps referred to as the urea cycle. Enzyme deficiencies in the urea cycle,
including ornithine transcarbamylase deficiency, result in the accumulation of ammonia
and precursor metabolites. This patient learned to avoid high protein foods in order
to prevent precipitation of physical symptoms brought on by the accumulation of these
toxic metabolites, resulting in protein malnutrition and cachexia. When parenteral
nutrition (with amino acids) was administered to this patient as treatment for pancreatitis,
accumulation of ammonia and other metabolites precipitated a seizure. An elevated
ammonia level is found in all urea cycle enzyme deficiencies, including ornithine
transcarbamylase deficiency. An ammonia level should be checked in all patients in
whom a urea cycle enzyme deficiency is considered.
WOOSHING IN MY EAR: I THINK IT SOUNDS LIKE LUPUS
E. Choe
1; H. Meatty1; D. Spruill1. 1Tulane Health Sciences Center, New Orleans, LA. (Tracking
ID #117444)
LEARNING OBJECTIVES
1. Recognize the presentation of lupus nephritis 2. Recognize the clinical presentation
of sagittal venous thrombosis.
CASE
A 19 year-old woman presented with two weeks of a headache associated with a “whooshing
sound” in her left ear. She noted associated epistaxis, but no fever, neck stiffness
or previous head trauma. She was afebrile and her vital signs were normal. She had
a resolving discoid rash on her abdomen, but the remaining examination was normal.
She had no focal neurologic deficits, and there were no carotid bruits. She had a
history of renal insufficiency, and her baseline creatinine of 1.8 had increased on
this admission to 6.4. Her hemoglobin was 4.0 g/dl; platelets 110. She had 250 blood
and >100 RBC on microscopic examination of the urine. Her ANA was positive at 1:640
and she had low C3 levels with a false positive RPR. Her PTT was elevated. She was
diagnosed with systemic lupus with renal failure secondary to lupus nephritis and
was treated with high dose pulse steroids andcyclophosphamide. Although a CT of the
head was normal, the persistent headache prompted an MRI with contrast that revealed
a sagittal vein thrombosis.
DISCUSSION
Renal impairment is a common presenting complaint of lupus, affecting 90% of patients
with lupus at some point in their course. Our patient had diffuse proliferative lupus
nephritis, the most common and the most severe form of disease. Aggressive therapy
with high dose pulse steroids and cyclophosphamide is indicated in these patients.
Lupus patients are also at risk for anti-phospholipid antibody syndrome inducing a
venous thrombosis. While deep venous thrombosis or stroke are the most common complications,
a wooshing sound in the ear in the setting of a headache is suggestive of either severe
carotid artery stenosis or venous thrombosis. In the latter diagnosis, the sound is
induced from turbulence created from venous back-pressure from the thrombosis on the
carotid artery flow. Fifty percent of lupus patients will have a CNS event of some
type during the lifetime.
WWW.WPW: WHAT WOULD WOLFE-PARKINSON-WHITE DO?
S. Kahlon
1; J. Wiese2. 1Tulane Health Sciences Center, New Orleans, LA; 2Tulane University,
New Orleans, LA. (Tracking ID #117478)
LEARNING OBJECTIVES
1. Review the symptomatic presentation and treatment of Wolf-Parkinson-White Syndrome.
2. Recognize the medications contraindicated in Wolf-Parkinson-White.
CASE
A 35 year-old man presented after an episode of palpitations, shortness of breath,
and an aching pain in the left upper chest and shoulder. He also noted lightheadedness
that occurred suddenly and at rest, resolving spontaneously after twenty minutes.
An ECG showed sinus rhythm with a rate of 83 BPM, left axis deviation, a QRS interval
of 0.104 seconds, a PR interval of 0.126 seconds, and delta waves. Electrolytes and
serial cardiac enzymes were normal. He was asymptomatic in the ER, and thus no treatment
was given. He was discharged to clinic with a prescription for a calcium channel blocker
to control the heart rate. At presentation in clinic, a repeat EKG was obtained demonstrating
similar intervals and the delta waves noted above. Owing to the risk of accelerated
conduction through the accessory pathway, the calcium channel blocker was held, and
the patient was referred to cardiology for ablation.
DISCUSSION
Wolf-Parkinson-White Syndrome has a prevalence of 1/500 people, making it one of the
more common congenital heart diseases. It is caused by an accessory electrical pathway
between the atria and ventricles leading to conduction abnormalities that can cause
arrhythmias. It is usually asymptomatic, discovered only by incidental EKG. When symptoms
do present, they usually take the form of ventricular tachycardia (70%), atrial fibrillation
(16%), or other arrhythmias. Recognizing the syndrome is important since treatment
with Class I and III antiarrhythmics or radioablation is available. ECG changes of
Wolf-Parkinson-White include the pathognemonic delta wave, a short PR interval (<0.12
seconds), and a widened QRS complex (>0.10 seconds) that are all manifestations of
early ventricular activation through the accessory pathway. The EKG is also diagnostic
of the location of the pathway: the left axis deviation in this case suggests that
the pathway is posterio-septal, readily amenable to radioablation from a right heart
cathertization. It is also important to recognize this syndrome to avoid administration
of digoxin and Class II and IV antiarrhythmics that may increase AV nodal blockade,
exacerbating the accessory pathway and potentially causing ventricular arrhythmias
and sudden death.
YES, YOU CAN HAVE TOO MUCH OF A GOOD THING: A CASE OF THE MILK ALKALI SYNDROME
G. Ramani
1; R. Granieri1. 1University of Pittsburgh, Pittsburgh, PA. (Tracking ID #116446)
LEARNING OBJECTIVES
1) Recognize milk alkali syndrome as an increasingly more common cause of hypercalcemia
2) Recognize the role of dietary calcium carbonate in the milk-alkali syndrome.
CASE
The patient is a 49 year old female, with past medical history of osteopenia with
recent shoulder and spine fractures, who presented with a three day history of increased
fatigue, nausea, and weakness. The patient had become very confused, fallen several
times, and was brought to the ED by her husband, who provided much of the history.
She had been very concerned about both her recent diagnosis of osteopenia and recent
heartburn, had been taking 100–250 Rolaids® (calcium carbonate, magnesium hydroxide)
tablets per week, as well as large quantities of baking soda (sodium bicarbonate).
Physical examination was notable for the patient being oriented only to name, and
for inappropriate speech. Reflexes, strength, cranial nerve testing, and sensation
were intact. Laboratory testing revealed a creatinine of 3.8, bicarbonate of 64, potassium
of 2.3, and a total serum calcium of 20. ABG demonstrated a pH of 7.55, pC02 of 60,
and pO2 of 99. EKG revealed no abnormalities. The patient was admitted to the hospital
and vigorously rehydrated with normal saline . Her electrolytes corrected over several
days. Her creatinine stabilized at 1.5, and her mental status improved. The patient
was educated about judicious calcium supplementation and discharged to home.
DISCUSSION
The milk-alkali syndrome is characterized by renal failure, hypercalcemia, and metabolic
alkalosis. Although once considered rare, increased awareness and screening for osteoporosis
has resulted in a marked rise in the number of patients taking calcium supplementation.
The pathophysiology is related to consumption of large quantities of calcium, in the
presence of an absorbable alkali. As calcium levels rise, serum levels of calcitriol
fall, thereby decreasing renal absorption of calcium. However, the ingestion of greater
then 10 grams of calcium can overwhelm this mechanism, and the presence of a metabolic
alkalosis can enhance renal absorption of calcium. Furthermore, hypercalcemia frequently
worsens the metabolic alkalosis by stimulating proton secretion and bicarbonate reabsorption
within the kidney. The diagnosis can usually be obtained by the history along with
documentation of electrolyte abnormalities. Treatment consists of cessation of the
offending agents, and rapid hydration with normal saline in patients with renal impairment
or severe hypercalcemia. Although renal function usually improves, some residual insufficiency
may persist. Prevention of this condition focuses upon educating patients of the importance
of limiting their supplemental calcium to no greater than 2 grams a day.