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

A. M. van Heyningen, M. J. Levenston, N. Tamminga, E. G. Scoop-Martijn, R. M. F. Wever, A. A. E. Verhagen, F. P. L. van der Dijs, D. A. J. Dijck-Brouwer, P. J. Offringa, F. A. J. Muskiet*

*Bijbehorende auteur voor dit werk

OnderzoeksoutputAcademicpeer review

3 Citaten (Scopus)


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.

Originele taal-2English
Pagina's (van-tot)301-304
Aantal pagina's4
TijdschriftWest Indian Medical Journal
Nummer van het tijdschrift4
StatusPublished - sep-2009

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