title: Neonatal screening for sickle cell disease.
abstract: BACKGROUND
  Sickle cell disease is an inherited disorder that occurs throughout the world with its highest incidence in areas of Africa where malaria is endemic. It affects up to one in 60 infants born in some areas of Africa. There are a number of potentially serious complications associated with the condition, and it is suggested that early treatment (before symptoms develop) can improve both morbidity and mortality. Screening for the condition in the neonatal period would enable early diagnosis and therefore early treatment.
  
  
  OBJECTIVE
  To assess whether there is evidence that neonatal screening for sickle cell disease rather than symptomatic diagnosis reduces adverse short and long term outcomes for those in whom the disease is detected, without adverse outcomes in the population screened.
  
  
  METHODS
  We searched the Controlled Trials Register of the Cochrane Cystic Fibrosis and Genetic Disorders Group (See Group search strategy). Contact was made with experts in the field for any work as yet unpublished and reference lists of published studies were also searched. Date of the most recent search of the Group's specialised register: November 1999.
  
  
  METHODS
  Any randomised or pseudorandomised trial, published or unpublished comparing diagnosis by screening to clinical diagnosis would have been considered eligible for inclusion.
  
  
  METHODS
  No trials of neonatal screening for sickle cell disease were found.
  
  
  RESULTS
  No trials of neonatal screening for sickle cell disease were found.
  
  
  CONCLUSIONS
  There is a lack of evidence from trials of neonatal screening for sickle cell disease. There is evidence of benefit from early treatment which is made possible by screening and there are a number of reviews and economic analyses of non-trial literature suggesting that screening is appropriate. Health care providers must therefore assess whether the information provided by these documents is relevant to their practice and situation when making decisions regarding neonatal screening for sickle cell disease. Systematic reviews of early treatments/interventions, including penicillin prophylaxis, pneumococcal vaccine and parental education should be considered.
authors.affiliation: Evidence Based Child Health Unit, Institute of Child Health, Royal Liverpool Children's Hospital, Eaton Rd, Liverpool, UK, L12 2AP. cmlees@liverpool.ac.uk
authors.forename: C M
authors.initials: CM
authors.lastname: Lees
authors.forename: S
authors.initials: S
authors.lastname: Davies
authors.forename: C
authors.initials: C
authors.lastname: Dezateux
mesh_terms: D000755:Anemia, Sickle Cell / Q000175:diagnosis*; D006801:Humans; D007231:Infant, Newborn; D015997:Neonatal Screening