Related Experiment Videos
Stroke in a cohort of patients with homozygous sickle cell disease
B Balkaran1, G Char, J S Morris
1Department of Child Health, University of the West Indies, Jamaica.
Insights
Children with homozygous sickle cell disease have a 7.8% risk of stroke by age 14. High white blood cell counts and sudden drops in hemoglobin are identified as key stroke risk factors.
Area of Science:
- Pediatric Neurology
- Hematology
- Vascular Neurology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with significant cerebrovascular complications.
- Homozygous sickle cell disease (HbSS) carries a high risk of stroke in childhood.
- Early identification of stroke risk factors is crucial for preventative strategies.
Observation:
- A cohort of 310 children with homozygous sickle cell disease was followed from birth.
- Stroke incidence was 7.8% by age 14, with 17 events recorded.
- Two subarachnoid hemorrhages and 15 presumed cerebral infarctions were observed.
Findings:
- Recurrent strokes occurred in 46% of initial survivors, with a median interval of 9 months.
- Elevated leukocyte counts were significantly higher in children with SCD and stroke compared to controls.
- Initial strokes were associated with acute decreases in hemoglobin (e.g., aplastic crisis, sequestration) and painful crises.
Implications:
- High leukocyte count and acute hemoglobin decrease are significant risk factors for stroke in homozygous sickle cell disease.
- These findings support closer monitoring and potential interventions in at-risk pediatric SCD patients.
- Further research into the mechanisms linking these hematologic markers to stroke is warranted.
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.