Sickle cell disease in children in Dakar, Senegal.

Aim of the study: to determine the socioeconomic, clinical and biological a spects 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). Results. - Homozygous sickle cell (SS) was the most frequent genotype (307 cases). Sickle cell hemoglobin C (13 cases) and sickle cell beta-thalassemi a (three cases) were uncommon. Patients were aged from five months to 22 ye ars (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%), Hae mophilus influenzae b (8.4%), Salmonella (8.7%) and Streptococcus pneumonia e (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 s uch as acute chest syndrome (1%), stroke (1%), cholelithiasis (9%), meningi tis (0.4%), septicemia (2%) and osteomyelitis (6%) were rare. Mean steady s tale hemoglobin (Hb) and hemoglobin F(HbF) levels were 8.27 +/- 1.36 g/dL a nd 6.8 +/- 5.9% respectively among SS patients. No correlations were found neither between Hb and HbF nor between these parameters and the frequency o f complications. Eleven patients (1.1% per year of follow-up) died, and inf ection was the main cause of death (73%). Conclusion. - In comparison with published data, SCD seems to have mild sev erity in Senegalese children and adolescents in spite of poor follow-up con ditions. In addition to genetic factors, environmental factors might have a n important role in disease tolerance. (C) 2000 Editions scientifiques et m edicales Elsevier SAS.