Stroke risk in siblings with sickle cell anemia

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Abstract
Patients with sickle cell disease who were younger than 21 years, including 2353 patients with HbSS. The stroke preva- lence was 4.9% for all genotypes; 7.1% for patients with HbSS; 1.1% for patients with HbSo thalassemia; 0.6% for pa- tients with S thalassemia; and 0% for patients with HbSC. In 207 sibships, more than 1 child had HbSS. There were 42 sibships in which at least 1 sibling had a stroke, and in 10 of the 42, 2 siblings had a stroke. A permutation test indicated that the number of families in which 2 children had strokes was larger than the number expected if strokes were randomly distrib- uted among children in sibships (P .0012). There was no difference in stroke prevalence based on sex, nor was the mean age at stroke presentation signifi- cantly different between singletons and sibships with stroke. We conclude that there is a familial predisposition to stroke in HbSS. Attempts to identify genetic modifiers should be initiated with family- based studies. (Blood. 2003;101:2401-2404)

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