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.
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 SCDpatients per 3 years in Aruba and 2 SCDpatients per year in St. Maarten. CONCLUSIONS: Universal screening of newborns for SCD seems cost-effective for St. Maarten.
Authors: Eline A Verberne; Ginette M Ecury-Goossen; Meindert E Manshande; Maria Ponson-Wever; Maartje de Vroomen; Martijn Tilanus; Marcel M A M Mannens; Lidewij Henneman; Mieke M van Haelst Journal: J Community Genet Date: 2021-03-10
Authors: Eline A Verberne; Ginette M Ecury-Goossen; Meindert E Manshande; Maria Ponson-Wever; Maartje de Vroomen; Martijn Tilanus; Marcel M A M Mannens; Lidewij Henneman; Mieke M van Haelst Journal: J Community Genet Date: 2021-03-10
Authors: Eline A Verberne; Jonne M Westermann; Tamar I de Vries; Ginette M Ecury-Goossen; Shirley M Lo-A-Njoe; Meindert E Manshande; Sonja Faries; Hans D Veenhuis; Patricia Philippi; Farah A Falix; Irsa Rosina-Angelista; Maria Ponson-Wever; Louise Rafael-Croes; Patricia Thorsen; Eric Arends; Maartje de Vroomen; Sietse Q Nagelkerke; Martijn Tilanus; Lars T van der Veken; Karin Huijsdens-van Amsterdam; Anne-Marie van der Kevie-Kersemaekers; Mariëlle Alders; Marcel M A M Mannens; Mieke M van Haelst Journal: Am J Med Genet A Date: 2022-03-07 Impact factor: 2.578
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