title: [Sickle cell disease in children in Dakar, Senegal].
abstract: OBJECTIVE
  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).
  
  
  RESULTS
  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%).
  
  
  CONCLUSIONS
  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.
authors.affiliation: L'université Cheikh-Anta-Diop de Dakar, hôpital d'enfants Albert-Royer, Sénégal.
authors.forename: I
authors.initials: I
authors.lastname: Diagne
authors.forename: O
authors.initials: O
authors.lastname: Ndiaye
authors.forename: C
authors.initials: C
authors.lastname: Moreira
authors.forename: H
authors.initials: H
authors.lastname: Signate-Sy
authors.forename: B
authors.initials: B
authors.lastname: Camara
authors.forename: S
authors.initials: S
authors.lastname: Diouf
authors.forename: A
authors.initials: A
authors.lastname: Diack-Mbaye
authors.forename: M
authors.initials: M
authors.lastname: Ba
authors.forename: M
authors.initials: M
authors.lastname: Sarr
authors.forename: D
authors.initials: D
authors.lastname: Sow
authors.forename: M
authors.initials: M
authors.lastname: Fall
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000191:economics / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004777:Environment; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D018570:Risk Assessment; D012675:Senegal; D012720:Severity of Illness Index; D012923:Social Class