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      First Presentation of Sickle Cell Anemia in A 3.5-Year-Old Girl: A Case Report

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          Abstract

          Dear Editor, Sickle cell disease is an inherited disorder of hemoglobin synthesis, which affects many individuals worldwide. Vaso-occlusive, aplastic and sequestration crises are common in a sickle cell child.[1] One of the leading causes of death in children with sickle cell anemia is an acute splenic sequestration crisis.[2] Children with splenic sequestration disease who have not yet undergone autosplenectomy, as well as older patients with sickle cell disease or S-beta thalassemia, may have sudden, rapid, massive enlargement of the spleen with trapping of a considerable portion of the red cell mass. On physical examination, there may be profound hypotension with cardiac decompensation and massive splenomegaly. The hemoglobin concentration is at least 2 g/dL lower than baseline and is accompanied by a brisk reticulocytosis with increased nucleated red cells and moderate to severe thrombocytopenia.[2] ACS is an acute illness with lung injury characterized by any combination of chest pain, fever, or respiratory symptoms and accompanied by a new pulmonary infiltrate on a chest radiograph.[3] Elevated WBC count, dactylitis, and anemia predicted more severe later outcomes in children younger than 4 years.[4] Dactylitis, often referred to as hand-foot syndrome, is frequently the 1st manifestation of pain in children with sickle cell anemia, occurring in 50% of children by 2 years of age.[5] A 3.5-year-old girl from race of Arab reffered to Shafa Hospital with severe anemia, thrombocytopenia, leucocytosis and elevated ESR and LDH. Her parents assigned fever, cough, pallor, weakness and tachypnea from six day ago (Figure 1). Fig. 1 Chest x ray. Past medical history and familial history of the patient was negative. On physical examination, she had fever with temperature of 39.5°C, severe pallor, pulse rate of 110 per minute, respiratory rate of 32 per minute and blood pressure of 90/60 mmHg. On abdominal examination, she had hepatosplenomegaly. Rales and rhonchi were in both lung fields on respiratory examination while the other systemic examination was essentially normal. Initial laboratory investigations demonstrated hemoglobin of 4.5 gm/dL, white cell count of 19,000/mm(3) (55% neutrophils, 45% lymphocyte), platelet count of 70,000/mm(3) and an erythrocyte sedimentation rate of 45 mm/hour. Renal function tests and urinalysis were normal. A chest radiograph revealed bilateral haziness. For decline of malignancy, bone marrow aspiration was done and discussed reactive bone marrow due to infection. After one day a dactylitis was present in hands of the patient (Figure 2). Fig. 2 Dactylitis. In follow up of Hb electrophoresis, Hb s was 80%, Hb F: 18%, and Hb A2: 2%. Finally in peripheral blood smear, sickling of RBC was detected and the patient was diagnosed with sickle cell anemia and acute splenic sequestration crisis which was associated with acute chest syndrome treated with wide spectrum antibiotic (cefotaxim and erythromycin) and transfusion exchange (Figure 3). The patient was discharged with stable clinical state after 8 days. Fig. 3 Prepheral blood smears with sickle cell. The clinical presentation of our patient is rare and unusual for initial presentation of sickle cell anemia. Also association between acute chest syndrome and splenic sequestration is unusual.

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          Prediction of adverse outcomes in children with sickle cell disease.

          The ability to identify infants with sickle cell anemia who are likely to have severe complications later in life would permit accurate prognostication and tailoring of therapy to match disease-related risks and facilitate planning of clinical trials. We attempted to define the features of such babies by following the clinical course of 392 children with sickle cell disease from infancy to about the age of 10 years. We analyzed the records of 392 infants who received the diagnosis of homozygous sickle cell anemia or sickle cell-Beta(0)-thalassemia before the age of six months and for whom comprehensive clinical and laboratory data were recorded prospectively; data were available for a mean (+/-SD) of 10.0+/-4.8 years. Results obtained before the age of two years were evaluated to determine whether they predicted the outcome later in life. Of the 392 infants in the cohort, 70 (18 percent) subsequently had an adverse outcome, defined as death (18 patients [26 percent]), stroke (25 [36 percent]) frequent pain (17 [24 percent]), or recurrent acute chest syndrome (10 [14 percent]). Using multivariate analysis, we found three statistically significant predictors of an adverse outcome: an episode of dactylitis before the age of one year (relative risk of an adverse outcome, 2.55; 95 percent confidence interval, 1.39 to 4.67), a hemoglobin level of less than 7 g per deciliter (relative risk, 2.47; 95 percent confidence interval, 1.14 to 5.33), and leukocytosis in the absence of infection (relative risk, 1.80; 95 percent confidence interval, 1.05 to 3.09). Three easily identifiable manifestations of sickle cell disease that may appear in the first two years of life (dactylitis, severe anemia, and leukocytosis) can help to predict the possibility of severe sickle cell disease later in life.
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            "Acute chest syndrome" in adults with sickle cell anemia. Microbiology, treatment, and prevention.

            Fifty-two episodes of fever, chest pain, increased leukocytosis, and pulmonary infiltrate ("acute chest syndrome") were studied in 28 adults with sickle cell anemia. Possible bacterial pathogens were identified in sputum cultures from less than half of the episodes; no pneumococci were found, and Staphylocococcus aureus was the only bacterium associated with a longer illness than that seen when only normal flora were recovered. Much disease diagnosed as "pneumonia" in adults with sickle cell anemia is probably pulmonary infarction. Many of these patients will recover with no more than modest supportive care; if antibiotics are used they should be directed against S aureus (and possibly Hemophilus species). Pneumococcal polysaccharide vaccine has great potential for preventing life-threatening infection in children with sickle cell anemia, but may not change the incidence or severity of the acute chest syndrome in adults.
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              Sickle cell disease in childhood: Part II. Diagnosis and treatment of major complications and recent advances in treatment.

              Treatment advances over the past 25 years have significantly decreased morbidity and mortality in children with sickle cell disease. Aggressive management of fever, early diagnosis of acute chest syndrome, judicious use of transfusions and proper treatment of pain can improve quality of life and prognosis for these children. Prophylactic hydroxyurea therapy has been shown to reduce the incidence and severity of pain crises in adults with sickle cell disease and has been effective in limited studies conducted in children. Research into stem cell transplantation provides hope that a cure for sickle cell disease may be possible.
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                Author and article information

                Journal
                Iran Red Crescent Med J
                Iran Red Crescent Med J
                Kowsar
                Iranian Red Crescent Medical Journal
                Kowsar
                2074-1804
                2074-1812
                March 2012
                01 March 2012
                : 14
                : 3
                : 184-185
                Affiliations
                [1 ]Thalassemia and Hemoglobinopathy Research Center, Ahvaz Jondishapur University of Medical Sciences, Ahvaz, Iran
                [2 ]Department of Pediatrics Hematology/Oncology, Ahvaz Jondishapur University of Medical Sciences, Ahvaz, Iran
                Author notes
                [* ]Correspondence: Mohammad Pedram, MD, Thalassemia and Hemoglobinopathy Research Center, Ahvaz Jondishapur University of Medical Sciences, Ahvaz, Iran. Tel.: +98-611-3743285, Fax: +98-611-3743285, E-mail: m_pedram_2007@ 123456yahoo.com
                Article
                3372033
                22737578
                2fbbe667-39cf-4736-8a72-18c567b18ae9
                Copyright © 2012, Kowsar Corp.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 12 August 2011
                : 25 November 2011
                Categories
                Letter to Editor

                Medicine
                dactylitis,splenic sequestration,sickle cell anemia
                Medicine
                dactylitis, splenic sequestration, sickle cell anemia

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