title: Burden of sickle cell trait and disease in the Uganda Sickle Surveillance Study (US3): a cross-sectional study.
abstract: BACKGROUND
  Sickle cell disease contributes substantially to mortality in children younger than 5 years in sub-Saharan Africa. In Uganda, 20,000 babies per year are thought to be born with sickle cell disease, but accurate data are not available. We did the cross-sectional Uganda Sickle Surveillance Study to assess the burden of disease.
  
  
  METHODS
  The primary objective of the study was to calculate prevalence of sickle cell trait and disease. We obtained punch samples from dried blood spots routinely collected from HIV-exposed infants in ten regions and 112 districts across Uganda for the national Early Infant Diagnosis programme. Haemoglobin electrophoresis by isoelectric focusing was done on all samples to identify those from babies with sickle trait or disease.
  
  
  RESULTS
  Between February, 2014, and March, 2015, 99,243 dried blood spots were analysed and results were available for 97,631. The overall number of children with sickle cell trait was 12,979 (13·3%) and with disease was 716 (0·7%). Sickle cell numbers ranged from 631 (4·6%) for trait and 23 (0·2%) for disease of 13,649 in the South Western region to 1306 (19·8%) for trait and 96 (1·5%) for disease of 6581 in the East Central region. Sickle cell trait was seen in all districts. The lowest prevalence was less than 3·0% in two districts. Eight districts had prevalence greater than 20·0%, with the highest being 23·9%. Sickle cell disease was less common in children older than 12 months or who were HIV positive, which is consistent with comorbidity and early mortality.
  
  
  CONCLUSIONS
  Prevalence of sickle cell trait and disease were high in Uganda, with notable variation between regions and districts. The data will help to inform national strategies for sickle cell disease, including neonatal screening.
  
  
  BACKGROUND
  Cincinnati Children's Research Foundation.
authors.affiliation: Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda.
authors.forename: Grace
authors.initials: G
authors.lastname: Ndeezi
authors.affiliation: Central Public Health Laboratories, Kampala, Uganda.
authors.forename: Charles
authors.initials: C
authors.lastname: Kiyaga
authors.affiliation: Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Arielle G
authors.initials: AG
authors.lastname: Hernandez
authors.affiliation: Mulago Hospital, Kampala, Uganda.
authors.forename: Deogratias
authors.initials: D
authors.lastname: Munube
authors.affiliation: Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Thad A
authors.initials: TA
authors.lastname: Howard
authors.affiliation: Central Public Health Laboratories, Kampala, Uganda.
authors.forename: Isaac
authors.initials: I
authors.lastname: Ssewanyana
authors.affiliation: Ministry of Health for the Republic of Uganda, Kampala, Uganda.
authors.forename: Jesca
authors.initials: J
authors.lastname: Nsungwa
authors.affiliation: Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda.
authors.forename: Sarah
authors.initials: S
authors.lastname: Kiguli
authors.affiliation: Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda.
authors.forename: Christopher M
authors.initials: CM
authors.lastname: Ndugwa
authors.affiliation: Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. Electronic address: russell.ware@cchmc.org.
authors.forename: Russell E
authors.initials: RE
authors.lastname: Ware
authors.affiliation: Ministry of Health for the Republic of Uganda, Kampala, Uganda.
authors.forename: Jane R
authors.initials: JR
authors.lastname: Aceng
mesh_terms: D000755:Anemia, Sickle Cell / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015995:Prevalence; D012805:Sickle Cell Trait / Q000453:epidemiology; D014454:Uganda / Q000453:epidemiology