Primary hemorrhagic stroke in children with sickle cell disease is associated with recent transfusion and use of corticosteroids.
OBJECTIVE
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.


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.
Strouse
John J
JJ
Division of Pediatric Hematology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. jstrous1@jhmi.edu
Hulbert
Monica L
ML
DeBaun
Michael R
MR
Jordan
Lori C
LC
Casella
James F
JF
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000009:adverse effects*; D000755:Anemia, Sickle Cell / Q000150:complications*; D016022:Case-Control Studies; D002543:Cerebral Hemorrhage / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D020521:Stroke / Q000209:etiology*; D013997:Time Factors; D065227:Transfusion Reaction
D000305:Adrenal Cortex Hormones