TITLE

Estimated Incidence of Sickle-Cell Disease in Aruba and St Maarten suggests Cost-effectiveness of a Universal Screening Programme for St Maarten

AUTHOR(S)
van Heyningen, A. M.; Levenston, M. J.; Tamminga, N.; Scoop-Martijn, E. G.; Wever, R. M. F.; Verhagen, A. A. E.; van der Dijs, F. P. L.; Dijck-Brouwer, D. A. J.; Offringa, P. J.; Muskiet, F. A. J.
PUB. DATE
September 2009
SOURCE
West Indian Medical Journal;Sep2009, Vol. 58 Issue 4, p301
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
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.
ACCESSION #
47333537

 

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