Risk Factors For Cerebral Vasculopathy In a Sickle Cell Disease Newborn Cohort

Children with sickle cell disease (SCD) have a significant vascular morbidity responsible of pain and stroke. They are at high risk of cerebral vasculopathy (CV) that can be detected in these children by transcranial Doppler. The aim of this study was to identify risk factors for CV using longitudinal data in the 375 newborn cohort of Robert Debre Pediatric Sickle Cell Referral Center. Among the 59 children presented CV, seven had a stroke. There was a total of 2677 patient-years of follow up. Overall, the incidence rate of CV was 2.20/100 patient-years (95% CI 1.64-2.76) and the incidence rate of stroke was 0.26/100 patient-years (95% CI 0.07-0.46). The cumulative risk of CV by age 14 years was 26.0% (95% CI 20.0%-33.3%).To take into account longitudinal data, risk factors of CV were assessed by a survival Cox model built in two steps. The final Cox multivariable analyze for CV retained five variables. Upper airways obstruction (HR 1.41 95%CI 1.00-1.98), bronchial obstruction (HR 2.13 95%CI 1.77-2.57), acute anemic event (HR 1.15 95%CI 1.01-1.32) and reticulocyte count (HR 1.41 per 20000/mm³ increase 95%CI 1.16-1.71) were independent risk factors whereas hydroxycarbamide therapy (HR 0.03 95%CI 0.01-0.35) was protective. Evaluation and specific treatment for symptoms of asthma or sleep apnea or nocturnal respiratory abnormalities could potentially reduce the risk of CV and stroke after treatment. Our findings suggest that hydroxycarbamide could protect from CV.