Published online November 1, 2006
PEDIATRICS Vol. 118 No. 5 November 2006, pp. 1916-1924 (doi:10.1542/peds.2006-1241)
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ARTICLE

Primary Hemorrhagic Stroke in Children With Sickle Cell Disease Is Associated With Recent Transfusion and Use of Corticosteroids

John J. Strouse, MDa, Monica L. Hulbert, MDb, Michael R. DeBaun, MD, MPHc, Lori C. Jordan, MDd and James F. Casella, MDa

a Division of Pediatric Hematology, Department of Pediatrics
d Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland
b Division of Pediatric Hematology/Oncology
c Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri

OBJECTIVES. Primary hemorrhagic stroke is an uncommon complication of sickle cell disease, with reported mortality rates of 24% to 65%. Most reported cases are in adults; little is known about its occurrence in children. Proposed risk factors include previous ischemic stroke, aneurysms, low steady-state hemoglobin, high steady-state leukocyte count, acute chest syndrome, and hypertransfusion. We performed a retrospective case-control study to evaluate risk and prognostic factors for primary hemorrhagic stroke among children with sickle cell disease.

PATIENTS AND METHODS. Case subjects (sickle cell disease and primary hemorrhagic stroke) and control subjects (sickle cell disease and ischemic stroke) were identified at 2 children’s hospitals from January 1979 to December 2004 by reviewing divisional records and the discharge databases.

RESULTS. We identified 15 case subjects (mean age: 10.4 ± 1.3 years) and 29 control subjects (mean age: 5.2 ± 0.4 years). An increased risk of hemorrhagic stroke was associated with a history of hypertension and recent (in the last 14 days) transfusion, treatment with corticosteroids, and possibly nonsteroidal antiinflammatory drugs. Average blood pressures at well visits (adjusted for age and gender) were similar between the 2 groups, suggesting that hypertension was intermittent

CONCLUSIONS. In this group of children with sickle cell disease, hemorrhagic stroke was associated with a history of hypertension or antecedent events including transfusion or treatment with corticosteroids. Improved understanding of risk and prognostic factors, especially those that are modifiable, may help prevent this devastating complication in children with sickle cell disease.

Key Words: sickle cell disease • cerebral hemorrhage • transfusions • stroke • case-control study

Abbreviations: SCD—sickle cell disease • HbSS—sickle cell anemia • ACS—acute chest syndrome • TIA—transient ischemic attack • OR—odds ratio • CI—confidence interval • NSAID—nonsteroidal antiinflammatory drug

Accepted Jun 20, 2006.


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