title: Stroke in a cohort of patients with homozygous sickle cell disease.
abstract: Strokes occurred in 17 of 310 children with homozygous sickle cell disease who were followed from birth, representing an incidence of 7.8% by the age of 14 years. Two children had subarachnoid hemorrhage, one having resolution of symptoms after aneurysm surgery and another dying of a presumed second hemorrhage 14 days later. The remaining 15 strokes were presumed to be cerebral infarction, although autopsy, angiographic, or computed tomographic evidence was available in only 8 children. There were 6 deaths, 2 in the acute event and 4 after recurrence, which occurred in 6 (46%) of 13 patients who survived the initial episode. There were 10 recurrent episodes at a median interval of 9 months after the initial event. Steady-state hematologic data revealed significantly higher leukocyte counts than in control subjects without strokes at age 1 year and in the last study preceding the stroke. The initial stroke coincided with an acutely lowered hemoglobin value in 5 patients (3 aplastic crises, 1 acute splenic sequestration, 1 probable pulmonary sequestration) and with painful crises in another 7 patients. We conclude that a high leukocyte count and an acute decrease of hemoglobin are risk factors for stroke in patients with homozygous sickle cell disease.
authors.affiliation: Department of Child Health, University of the West Indies, Jamaica.
authors.forename: B
authors.initials: B
authors.lastname: Balkaran
authors.forename: G
authors.initials: G
authors.lastname: Char
authors.forename: J S
authors.initials: JS
authors.lastname: Morris
authors.forename: P W
authors.initials: PW
authors.lastname: Thomas
authors.forename: B E
authors.initials: BE
authors.lastname: Serjeant
authors.forename: G R
authors.initials: GR
authors.lastname: Serjeant
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000235:genetics; D001772:Blood Cell Count; D002561:Cerebrovascular Disorders / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006429:Hemiplegia / Q000209:etiology; D006454:Hemoglobins / Q000032:analysis; D006720:Homozygote; D006801:Humans; D007223:Infant; D008297:Male; D012307:Risk Factors
chemical_list: D006454:Hemoglobins