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

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.
September 2009
West Indian Medical Journal;Sep2009, Vol. 58 Issue 4, p301
Academic Journal
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.


Related Articles

  • Hemoglobin A2 values in sickle cell disease patients quantified by high performance liquid chromatography and the influence of alpha thalassemia. da Fonseca, Silvana Fahel; Amorim, Tatiana; Purificação, Antônio; Gonçalves, Marilda; Boa-Sorte, Ney // Revista Brasileira de Hematologia e Hemoterapia;Sep/Oct2015, Vol. 37 Issue 5, p296 

    Background: In sickle cell disease, the quantification of Hb A2 is important for the differential diagnosis between sickle cell anemia (Hb SS) and Hb S/(β°-thalassemia. Objective: To determine Hb A2 levels as quantified by high performance liquid chromatography in patients with sickle cell...

  • Implementing neonatal screening for haemoglobinopathies in the Netherlands. Bouva, Marelle J.; Mohrmann, Karin; Brinkman, Henri B. J. M.; Kemper-Proper, Evelien A.; Elvers, Bert; Loeber, J. Gerard; Verheul, Francesco E. A. M.; Giordano, Piero C. // Journal of Medical Screening;Jun2010, Vol. 17 Issue 2, p58 

    Background The birth prevalence of severe haemoglobinopathies such as sickle cell disease (SCD) in the Netherlands has been estimated to be at least 50 newborns per year. Neonatal screening for SCD was added to the Dutch screening programme in January 2007. We here evaluated three high...

  • Chromatographic analysis of Hb S for the diagnosis of various sickle cell disorders in Pakistan. Hashmi, Nazish Khalid; Moiz, Bushra; Nusrat, Maliha; Hashmi, Mashhooda Rasool // Annals of Hematology;Aug2008, Vol. 87 Issue 8, p639 

    Sickle cell disease remains a relatively obscure theme in research on haemoglobinopathies in Pakistan. Limited data is available regarding its prevalence in the country. The objective of our study was not only to estimate the frequency of different sickle cell diseases but also to provide...

  • HPLC analysis of Sickle cell disorders and role of fetal hemoglobin on various manifestations of the disease. Rainchwar, Sujay // New Indian Journal of Surgery;Oct2011, Vol. 2 Issue 4, p313 

    Objectives Recognizing the prevalence of sickle cell anemia in Yavatmal district, the study was carried out to find out distribution of the disorder in Yavatmal and to study the extent of various manifestations in homozygous sickle cell cases & correlate them with fetal hemoglobin levels using...

  • Negative Epistasis between Sickle and Foetal Haemoglobin Suggests a Reduction in Protection against Malaria. Mmbando, Bruno P.; Mgaya, Josephine; Cox, Sharon E.; Mtatiro, Siana N.; Soka, Deogratias; Rwezaula, Stella; Meda, Elineema; Msaki, Evarist; Snow, Robert W.; Jeffries, Neal; Geller, Nancy L.; Makani, Julie // PLoS ONE;May2015, Vol. 10 Issue 5, p1 

    Background: Haemoglobin variants, Sickle (HbS) and foetal (HbF) have been associated with malaria protection. This study explores epistatic interactions between HbS and HbF on malaria infection. Methods: The study was conducted between March 2004 and December 2013 within the sickle cell...

  • Hemoglobinopathies in South Gujarat population and incidence of anemia in them. Patel, Ankur G.; Shah, Avani P.; Sorathiya, Smita M.; Gupte, Snehalata C. // Indian Journal of Human Genetics;Sep-Dec2012, Vol. 18 Issue 3, p294 

    OBJECTIVE: To Screen of South Gujarat population for determination of prevalence of different hemoglobinopathies particularly beta thalassemia trait (BTT) and sickle cell trait (SCT) and find out the incidence of anemia in them. MATERIAL AND METHODS: The present study screened 32,857 samples of...

  • Changes in the High-Performance Liquid Chromatography Pattern of Hemoglobin Lepore After Treatment With Hydroxyurea. Nussenzveig, Roberto H.; Vanhille, Derek L.; Hussey, Dottie; Archana M. Agarwal // Laboratory Medicine;Summer2013, Vol. 44 Issue 3, p245 

    Hemoglobin (Hb) Lepore is a β+-thalassemic hemoglobin variant composed of 2 α-globin and 2 δβ-globin hybrid chains. Three common Hb Lepore variants have been identified based on the primary sequence of the fusion proteins. In most cases, diagnosis of Hb S/Hb Lepore compound...

  • Fetal hemoglobin & liver dysfunction in sickle cell crisis. Panigrahi, Sumanta; Sablania, Praveen; Khodiar, P. K.; Keshari, J. R.; Patra, Pradeep Kumar // Indian Journal of Public Health Research & Development;Jan-Jun2011, Vol. 2 Issue 1, p82 

    Fetal hemoglobin is one of the major factors that alters the clinical course of disease. A study of 45 patients of sickle cell crisis was carried out in Department of Biochemistry & Department of Pediatrics, Pt JNM Medical College, Raipur, Chhattisgarh between June 2007 to July 2009. All...

  • Modulating Effect of the -158 Gγ (C→T) Xmn1 Polymorphism in Indian Sickle Cell Patients. Pandey, Sanjay; Pandey, Sweta; Mishra, Rahasya Mani; Saxena, Renu // Mediterranean Journal of Hematology & Infectious Diseases;2012, Vol. 4 Issue 1, p1 

    Xmn1 polymorphism is a known factor, which increases fetal haemoglobin production. Among the inherited disorders of blood, thalassaemia and Sickle Cell Diseases contributes to a major bulk of genetic diseases in India. Our aim was to verify the role of the Xmn1 polymorphism as a modulating...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics