Rates of Strokes in Californians with Sickle Cell Disease in the Post-STOP Era.

Neurovascular complications, including strokes and transient ischemic attacks (TIAs), are common and cause significant morbidity in individuals with sickle cell disease (SCD). The STOP trial (1998) established chronic transfusions as the standard of care for children with SCD at high risk for stroke. Using statewide emergency department and hospitalization data from the California Department of Health Care Access and Innovation (1991-2019), we determined the cumulative incidence and rates for primary and recurrent strokes and TIAs in people with SCD pre- and post-STOP. For the 7,636 patients included in our SCD cohort, cumulative incidence for first ischemic stroke was 2.1% (95% CI: 1.8%, 2.4%) by age 20 years and 13.5% (95% CI: 12.3%, 14.7%) by age 60 years. Cumulative incidence for first intracranial hemorrhage (ICH) was 0.5% (95% CI: 0.4%, 0.7%) by age 20 years and 6.8% (95%CI: 5.9%, 7.7%) by age 60 years. Ischemic stroke rates increased in children (age <18 years, 234.9 vs 165.1 per 100,000PY, P=0.012) and adults (ages 31-50 years, 431.1 vs 303.2 per 100,000PY, P=0.031) in 2010-2019, compared to the preceding decade. There was an increase in rates of ICH in ages 18-30 and TIA in children <18 years from 2010-2019 when compared to the prior decade. Risk factors for stroke and TIA included increasing age, hypertension, and hyperlipidemia. These findings underscore the need for prevention of strokes in adults with SCD and suggest an emphasis on the management of modifiable cerebrovascular risk factors that have been demonstrated to be effective in the general population.

Cognitive functioning in children from Nigeria with sickle cell anemia.

BACKGROUND: Cognitive impairment is a major neurological complication of sickle cell anemia (SCA) in the United States, but there are limited studies of cognitive impairment in Nigeria, the country with the highest SCA burden. We hypothesized that children from Nigeria with SCA have worse cognitive functioning than comparison children and explored the association between lower cognitive functioning and key laboratory demographic and socioeconomic variables among children with SCA. PROCEDURE: We conducted a cross-sectional survey, supplemented by anthropomorphic and laboratory data, among a convenience sample of children from Nigeria with and without SCA. We administered the Wechsler Intelligence Scale for Children, Version IV. Our primary outcome measures included (1) estimated IQ (Est. IQ), (2) working memory (WM), and (3) processing speed (PS). RESULTS: The sample included 56 children with SCA (mean age 9.20 [SD 2.75], 46.43% girls) and 44 comparison children (mean age 9.41 [SD 2.49], 40.91% girls). Children with SCA performed worse on Est. IQ (84.58 vs. 96.10, P = 0.006) and PS (86.69 vs 96.91, P = 0.009) than comparison children. There was no significant difference in WM between both groups. Factors associated with lower Est. IQ and PS among children with SCA included age, maternal education, weight-for-age Z scores, and height-for age Z scores. CONCLUSION: In this small sample of children from Nigeria, we found worse cognitive functioning in children with SCA than in comparison children, and that sociodemographic and anthropomorphic factors were correlated with cognitive functioning.