Stroke in a cohort study of patients with homozygous sickle-cell disease - abstract

ABSTRACT

The features of 16 strokes occurring in a cohort of 310 children with homozygous sickle-cell (SS) disease followed from birth have been reviewed. The incidence was 6.9 percent by the age of 14 years. Recurrences occurred in 7/15 (47 percent) subjects surviving the first attack. Of the 14 recurrences 13 were within two years of the initial episode and the median interval was 9 months. Six children died, one during the initial episode and 5 during recurrences. The pathological basis was subarachnoid haemorrhage in 2, infarction in 7 and unknown in 7. The stroke group had significantly lower red cell counts and higher total nucleated counts in their preceding steady state haematology. The stroke was associated with an acute lowering of haemoglobin level in 5 patients (3 aplastic crises, 1 acute splenic sequestration, 1 unexplained), and with painful crises in 5 patients. These observations are the first in a representative sample of patients followed from birth and indicate a lower incidence of initial stroke and of recurrence than presented in other reports. The association with acute anaemia and with painful crisis has not been previously recognised, and suggests new directions for research in this complication (AU)