Sickle Cell Anemia Research - Genetics, Causes, Symptoms

Sickle Cell Anemia Research Today is a free monthly online journal that collates and summarizes the latest research about Sickle Cell Anemia, including details on genetics, causes, symptoms.


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Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease.

Strouse JJ, Takemoto CM, Keefer JR, Kato GJ, Casella JF

Division of Pediatric Hematology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. jstrous1@jhmi.edu

BACKGROUND: Acute chest syndrome (ACS) is a frequent cause of hospitalization and mortality in children with sickle cell disease. Transfusion is often required to prevent respiratory failure and treatment with dexamethasone may reduce the length of admission and the need for transfusions. We performed a retrospective cohort study to evaluate risk factors for readmission and prolonged hospitalization after different treatments for ACS. PROCEDURE: We identified patients <22 years of age hospitalized with ACS at Johns Hopkins Hospital from January 1998 to April 2004 using the hospitals discharge database and by reviewing dictated summaries. RESULTS: We identified 65 patients with 129 episodes of ACS (mean age 12.5 years, range 1.2-21.9 years). Thirty-nine episodes were treated with corticosteroids and 51 with transfusions. Patients were readmitted within 14 days after 23 episodes (18%). Readmission was strongly associated with report of an inhaler or nebulizer at home [odds ratio (OR) 6.0, P < 0.05], diastolic BP at 48 hr (OR 1.8 per 10 mm increase, P<0.01), corticosteroids (OR 20, P < 0.005), or transfusion (OR 0.03, P < 0.05). Treatment with corticosteroids alone (P < 0.05) and older age (P < 0.001) were associated with longer hospitalization. CONCLUSIONS: These results demonstrate a greatly elevated independent risk of readmission after ACS in children with asthma and after treatment with corticosteroids and a protective effect of transfusion. Although dexamethasone has documented efficacy for reducing the duration of ACS, the substantial risk of readmission for pain should limit its use.

Published 2 April 2008 in Pediatr Blood Cancer, 50(5): 1006-12.
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Sickle Cell Anemia Research Today Archive:

Volume 1 (2006)
  Issue 1 (February)
  Issue 2 (March)
  Issue 3 (April)
  Issue 4 (May)
  Issue 5 (June)
  Issue 6 (July)
  Issue 7 (August)
  Issue 8 (September)
  Issue 9 (October)
  Issue 10 (November)
  Issue 11 (December)

Volume 2 (2007)
  Issue 1 (January)
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  Issue 3 (March)
  Issue 4 (April)
  Issue 5 (May)
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Volume 3 (2008)
  Issue 1 (January)
  Issue 2 (February)
  Issue 3 (March)
  Issue 4 (April)
  Issue 5 (May)
  Issue 6 (June)
  Issue 7 (July)
  Issue 8 (August)



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