title: High rate of sickle cell anaemia in Sub-Saharan Africa underlines the need to screen all children with severe anaemia for the disease.
abstract: OBJECTIVE
  Neonatal screening for sickle cell anaemia is not common practice in the Democratic Republic of Congo, and we determined the prevalence in children with unknown electrophoresis of haemoglobin and anaemia.
  
  
  METHODS
  A cross-sectional study was conducted in four hospitals in the country's capital Kinshasa.
  
  
  RESULTS
  We screened 807 patients with anaemia (Hb < 6 g/dL) for sickle cell disease. The overall mean age at presentation was 42.7 months ± 29.7 months, and most patients (76.3%) were less than five years of age, with a peak incidence at seven to 36 months of age (45%). The median age at the first transfusion was 29 months (range 4-159 months). Of these 807 children, 36 (4.5%) were homozygous for haemoglobin S disease and 45 (5.6%) were heterozygotes. The proportion of patients with homozygous sickle cell anaemia was slightly higher in children with a medical history of hand foot syndrome, in children who had received more than three transfusions and in children up to 36 months of age at their first transfusion.
  
  
  CONCLUSIONS
  The high prevalence of sickle cell anaemia in children in Sub-Saharan Africa underlines the need for neonatal screening or, if that is not possible, screening of all children with severe anaemia to identify patients with the disease and provide early management.
authors.affiliation: Division of Hemato-oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Congo.
authors.forename: Bertin Tshimanga
authors.initials: BT
authors.lastname: Kadima
authors.affiliation: Division of Hemato-oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Congo.
authors.forename: Jean Lambert
authors.initials: JL
authors.lastname: Gini Ehungu
authors.affiliation: Division of Hemato-oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Congo.
authors.forename: René Makwala
authors.initials: RM
authors.lastname: Ngiyulu
authors.affiliation: Division of Hemato-oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Congo.
authors.forename: Pépé Mfutu
authors.initials: PM
authors.lastname: Ekulu
authors.affiliation: Division of Hemato-oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Congo.
authors.forename: Michel Ntetani
authors.initials: MN
authors.lastname: Aloni
mesh_terms: D017954:Africa South of the Sahara / Q000453:epidemiology; D000740:Anemia / Q000209:etiology*; D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008403:Mass Screening; D015995:Prevalence; D011446:Prospective Studies
keywords: Democratic Republic of Congo; Screening; Severe anaemia; Sickle cell anaemia; Sub-Saharan Africa