Les syndromes drépanocytaires majeurs en pédiatrie à Dakar (Sénégal)

Abstract

To determine the socioeconomic, clinical and biological aspects of sickle cell disease (SCD) in Senegalese children and adolescents, we retrospectively analysed all records of follow-up attending patients in the Albert Royer Children Hospital of Dakar (Senegal). Homozygous sickle cell (SS) was the most frequent genotype (307 cases). Sickle cell hemoglobin C (13 cases) and sickle cell beta-thalassemia (three cases) were uncommon. Patients were aged from five months to 22 years (mean age: eight years). Most of them came from poor families. The mean number of children was five in patients' families, with at least two cases of SCD in 60% of them. Immunization against hepatitis B virus (10.2%), Haemophilus influenzae b (8.4%), Salmonella (8.7%) and Streptococcus pneumoniae (21.4%) was insufficiently performed, because of its relatively high cost. Only 30% of the patients had received a blood transfusion. Painful crises occurred less than three times a year in 74% of the cases. Complications such as acute chest syndrome (1%), stroke (1%), cholelithiasis (9%), meningitis (0.4%), septicemia (2%) and osteomyelitis (6%) were rare. Mean steady state hemoglobin (Hb) and hemoglobin F(HbF) levels were 8.27 +/- 1.36 g/dL and 6.8 +/- 5.9% respectively among SS patients. No correlations were found neither between Hb and HbF nor between these parameters and the frequency of complications. Eleven patients (1.1% per year of follow-up) died, and infection was the main cause of death (73%). In comparison with published data, SCD seems to have mild severity in Senegalese children and adolescents in spite of poor follow-up conditions. In addition to genetic factors, environmental factors might have an important role in disease tolerance.