Epidemiology of sickle cell disease in Tunisia

Sickle cell disease is a serious health problem in Tunisia because of its high frequency and severity. We reported an epidemiological study on 32 patients with a major sickle cell syndrome. They were recruited from Paediatric units of central Tunisia Hospitals from 1996 till 2002. The diagnosis relied on epidemiological, clinical, biological and molecular features. Consanguinity prevalence was high (50%). Clinical manifestations were based on paleness and splenomegaly. Complications included painful crises, infections, severe anemia, vascular necrosis and chronic complications as biliary lithiasis. The manifestations have been more severe in SSdisease and less in SC forms. Patients have to be taken in charge as earlier as possible. The best treatment is allogenic bone marrow transplant; the preventive one is prenatal diagnosis by DNA analysis for couples at risk