Sickle Cell Disease in Mauritania: epidemiological, clinical and therapeutic aspects about 135 cases.

objectiveTo describe the epidemiological, clinical and therapeutic characteristics of a series of sickle cell disease cases collected in Mauritania over a three-year period.

methodsThis is a descriptive study of the profile of sickle cell disease, diagnosed in Mauritania, from January 1, 2015 to December 31, 2017, at the outpatient clinics of the National Hospital Center and at the Mauritanian Association for the Support of Sickle Cell Patients. Patients were included following diagnostic confirmation by hemoglobin electrophoresis.

resultsDuring the study period, a total of 135 patients were included (79 female and 56 male), i.e. a sex ratio of 0.7 and an average age of 24 years (extremes: 9 months -77 years). All Mauritanian ethnic groups were affected by sickle cell disease, mainly the Peulths (63.7%). Sickle cell disease was found in eight wilayas, particularly Brakna (39%). The age of diagnosis was between 3 and 5 years, in 48% of patients. Sickle cell disease was discovered at the stage of complications in twelve patients. There are three types of sickle cell phenotypes: SS (54%), AS (40%) and SC (6%). In addition to transfusion, preventive treatment consisted of folic acid (n=53), hydroxyurea (n=14), and long-term antibiotic prophylaxis (n=3).

conclusionThe profile of sickle cell disease in Mauritania remains attributed to the lack of an active screening strategy and rapid diagnosis, hence the importance of developing a national program for early detection and management.