261: Endothelial function in children with sickle cell disease

Abstract

Sickle cell anemia (SCA) is an inherited disorder characterized by recurrent painful crises with ischemia resulting from vascular occlusion. Reduction of the flow mediated dilatation (FMD), secondary to impaired release of substances such as nitric oxide, has been reported recently in adult patient with SCA, but little is known in childrens. (1) To describe the vascular abnormalities assessed carotid and brachial arteries by ultrasound in a population of children with a major sickle cell syndrome (SS). (2) To compare with controls (children of the same age with no cardiovascular risk factor). (3) To compare vascular parameters with clinical, biological and transcranial Doppler data. Thirty two patients with SCA were prospectively enrolled. Mean age was 12,3±4,5 years. Patients with SCA were closely matched for age, gender, to 50 healthy AA subjects. Carotid IMT, cross sectional compliance (CSC), distensibility (CSD) and FMD were determined non-invasively and compared among the two groups. FMD was significantly decreased in SCA patients vs controls (6,2±2,9 vs 8, 2±4%, p<0,05). Carotid IMT (0,45±0,03), CSC, CSD was comparable in SCA and controls. There was no correlation between FMD and age, number and severity of crises, hemoglobin and LDH level, transcranial Doppler. This study demonstrated endothelial dysfunction in children with SCA which could influence therapies in this population to prevent cardiovascular risk factor.