title: Estimated incidence of sickle-cell disease in Aruba and St. Maarten suggests cost-effectiveness of a universal screening programme for St. Maarten.
abstract: OBJECTIVE
  To estimate the incidence of Sickle-Cell Disease (SCD) in Aruba and St. Maarten and to determine whether universal screening would be cost-effective according to United Kingdom criteria.
  
  
  METHODS
  Consecutive cord blood samples were collected in Aruba and the Dutch part of St. Maarten during 3 and 4 months, respectively. Samples were subjected to High Performance Liquid Chromatography (HPLC) screening of haemoglobin variants.
  
  
  RESULTS
  Of the 368 samples (87.6% of all registered births) collected in Aruba, 10 (2.72%; CI 1.3, 4.9%) tested heterozygous for the Sickle-cell gene (HbAS) and 7 (1.90%; CI 0.8, 3.9%) for the haemoglobin C gene (HbAC). Of the 193 samples (83.5%) collected in St. Maarten, 14 (7.25%; CI 4.0, 11.9%) contained HbAS and 10 (5.18%; CI 2.5, 9.3%) HbAC. Hardy-Weinberg equilibrium predicted an incidence of 2.65% for HbAS and 1.86% for HbAC in Aruba and 6.80% for HbAS and 4.86% for HbAC in St. Maarten. These figures imply a newborn rate of about 2 SCD patients per 3 years in Aruba and 2 SCD patients per year in St. Maarten.
  
  
  CONCLUSIONS
  Universal screening of newborns for SCD seems cost-effective for St. Maarten.
authors.affiliation: Laboratory Medicine, University Medical Centre Groningen, The Netherlands.
authors.forename: A M
authors.initials: AM
authors.lastname: van Heyningen
authors.forename: M J
authors.initials: MJ
authors.lastname: Levenston
authors.forename: N
authors.initials: N
authors.lastname: Tamminga
authors.forename: E G
authors.initials: EG
authors.lastname: Scoop-Martijn
authors.forename: R M F
authors.initials: RM
authors.lastname: Wever
authors.forename: A A E
authors.initials: AA
authors.lastname: Verhagen
authors.forename: F P L
authors.initials: FP
authors.lastname: van der Dijs
authors.forename: D A J
authors.initials: DA
authors.lastname: Dijck-Brouwer
authors.forename: P J
authors.initials: PJ
authors.lastname: Offriinga
authors.forename: F A J
authors.initials: FA
authors.lastname: Muskiet
mesh_terms: D000755:Anemia, Sickle Cell / Q000191:economics / Q000453:epidemiology*; D003362:Cost-Benefit Analysis; D006801:Humans; D007231:Infant, Newborn; D015997:Neonatal Screening / Q000191:economics*; D014900:West Indies / Q000453:epidemiology