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      Fibromuscular dysplasia presenting as a renal infarction: a case report

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          Abstract

          Introduction

          Fibromuscular dysplasia is a non-atherosclerotic, non-inflammatory disease that most commonly affects the renal and internal carotid arteries.

          Case presentation

          We present the case of a 44-year-old Caucasian man who was admitted with complaints of loin pain and hypertension. A computed tomography scan of the abdomen revealed a right renal infarction with a nodular aspect of the right renal artery. Subsequent renal angiography revealed a typical 'string of beads' pattern of the right renal artery with thrombus formation. Oral anticoagulation was started and the secondary hypertension was easily controlled with anti-hypertensive drugs. At follow-up, our patient refused percutaneous transluminal renal angioplasty as a definitive treatment.

          Conclusions

          Fibromuscular dysplasia is the most common cause of renovascular hypertension in patients under 50 years of age. Presentation with renal infarction is rare.

          In fibromuscular dysplasia, angioplasty has been proven to have, at least for some indications, an advantage over anti-hypertensive drugs. Therefore, hypertension secondary to fibromuscular dysplasia is the most common cause of curable hypertension.

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          Most cited references20

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          Acute renal embolism. Forty-four cases of renal infarction in patients with atrial fibrillation.

          Acute renal embolus is rarely reported in the medical literature; thus, accurate data regarding presentation, laboratory tests, diagnostic techniques, and treatment are lacking. To better define this condition, we examined the medical records of all patients admitted to Kaplan Medical Center and Sheba Medical Center in central Israel from 1984 to 2002 who had a diagnosis of renal infarction and atrial fibrillation. We noted demographic, clinical, and laboratory parameters; method of diagnosis; treatment received; and patient outcome. We identified 44 cases of renal embolus: 23 females and 21 males, with an average age of 69.5 +/- 12.6 years. Thirty (68%) patients had abdominal pain, and 6 (14%) had a previous embolic event. Nine patients were being treated with warfarin on admission, 6 (66%) of whom had an international normalized ratio (INR) 400 U/dL. The mean LDH was 1100 +/- 985 U/dL. Diagnostic techniques included renal isotope scan, which was abnormal in 36/37 cases (97%); contrast-enhanced computed tomography (CT) scan, which was diagnostic in 12/15 cases (80%); and ultrasound, which was positive in only 3/27 cases (11%). Angiography was positive in 10/10 cases (100%). Twenty-three (61%) of 38 patients had normal renal function on follow-up. The 30-day mortality was 11.4%. Renal embolus was diagnosed mainly in patients aged more than 60 years, some of whom had a previous embolic event. Most of those receiving anticoagulant therapy had a subtherapeutic INR. Abdominal pain was common, as well as hematuria and an elevated LDH. These patients are at risk of subsequent embolic events to other organs. The most sensitive diagnostic technique in this population is a renal isotope scan, but contrast-enhanced CT scan requires further assessment. Copyright 2004 Lippincott Williams & Wilkins
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            Acute renal infarction. Clinical characteristics of 17 patients.

            We analyzed the medical records of patients with an established diagnosis of acute renal infarction to identify predictive parameters of this rare disease. Seventeen patients (8 male) who were admitted to our emergency department between May 1994 and January 1998 were diagnosed by contrast-enhanced computed tomography (CT) as having acute renal infarction (0.007% of all patients). We screened the records of the 17 patients for a history with increased risk for thromboembolism, clinical symptoms, and urine and blood laboratory results known to be associated with acute renal infarction. A history with increased risk for thromboembolism with 1 or more risk factors was found in 14 of 17 patients (82%); risk factors were atrial fibrillation (n = 11), previous embolism (n = 6), mitral stenosis (n = 6), hypertension (n = 9), and ischemic cardiac disease (n = 7). All patients reported persisting pain predominantly from the flank (n = 11), abdomen (n = 4), and lower back (n = 2). On admission, elevated serum lactate dehydrogenase was found in 16 (94%) patients, and hematuria was found in 12 (71%) of 17 patients. After 24 hours all patients showed an elevated serum lactate dehydrogenase, and 14 (82%) had a positive test for hematuria. Our findings suggest that in all patients presenting with the triad--high risk of a thromboembolic event, persisting flank/abdominal/lower back pain, elevated serum levels of lactate dehydrogenase and/or hematuria within 24 hours after pain onset--contrast-enhanced CT should be performed as soon as possible to rule out or to prove acute renal infarction.
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              Renal artery embolism: clinical features and long-term follow-up of 17 cases.

              Spontaneous renal artery embolism is not rare, but a correct diagnosis and appropriate treatment are often delayed. Clinical features and follow-up of 17 cases are reported. Cardiac disease or arrhythmias pre-existed in 16 patients. Initial symptoms included flank pain (seven cases), abdominal or chest pain alone (seven), and nausea and vomiting (eight). Fever (greater than or equal to 37.5 degree C) occurred in 10 cases and flank tenderness in only eight. Laboratory findings included leukocytosis, proteinuria, hematuria, and elevated levels of lactic dehydrogenase, serum glutamic-oxalacetic transaminase, serum glutamic-pyruvic transaminase, and alkaline phosphatase. Serum creatinine level exceeded 1.3 mg/dl in 88% and 4.0 mg/dl in 65%; four patients required dialysis. The diagnosis, made by scintiscan, arteriography, or both was often delayed. Renal embolization was bilateral in seven patients and unilateral in 10, with serum creatinine level above 4.0 mg/dl in five of the latter. Emboli to other organs caused early death; cardiovascular disease led to later death. With anticoagulants, renal function returned in patients surviving more than 1 month, even those with bilateral emboli. Thus, renal embolism is recognizable if the disease is considered, and a favorable outcome is common with long-term anticoagulants.
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                Author and article information

                Journal
                J Med Case Reports
                Journal of Medical Case Reports
                BioMed Central
                1752-1947
                2010
                30 June 2010
                : 4
                : 199
                Affiliations
                [1 ]Department of Nephrology-Hypertension, Antwerp University Hospital, Edegem (Antwerpen), Belgium
                [2 ]Department of Radiology, Antwerp University Hospital, Edegem (Antwerpen), Belgium
                [3 ]Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Belgium
                Article
                1752-1947-4-199
                10.1186/1752-1947-4-199
                2905432
                20591148
                82598e19-2c2b-4205-8de5-6d04981c66e4
                Copyright ©2010 Van den Driessche et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 January 2009
                : 30 June 2010
                Categories
                Case Report

                Medicine
                Medicine

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