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Table of Contents
Vol. 100, No. 1, 1998
Issue release date: July 1998
Section title: Original Paper
Acta Haematol 1998;100:49–53
(DOI:10.1159/000040863)

α-Thalassemia in the United Arab Emirates

El-Kalla S. · Baysal E.
Department of Pediatrics and Genetics and Department of Health and Medical Services, Dubai, United Arab Emirates

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Published online: August 05, 1998
Issue release date: July 1998

Number of Print Pages: 5
Number of Figures: 0
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA

Abstract

A neonatal screening survey of α-thalassemia (α-thal) among the United Arab Emirates (UAE) nationals was conducted on 418 consecutive cord blood samples. Our findings demonstrate that 49% of the cases studied were found with an α-globin gene defect. The gene frequency of the –α3.7 was 0.2847 and that of the –α4.2 was 0.0072. Four nondeletional α-thal mutations were found; αPA-1, αPA-2, Hb CS and α-5nt del with gene frequencies of 0.0036, 0.0012, 0.0024, and 0.0072, respectively. We also report here the genotype-phenotype correlation in 22 patients with Hb H disease or Hb H-like syndrome. Of these, 6 were homozygous for the αPA-1 mutation, 2 were homozygous for Hb CS, and 14 were compound heterozygous for either αPA-1, Hb CS, α-5nt del or ––MED-I, with the –α3.7. The data reported here demonstrate that a considerable heterogeneity of α-thal mutations occurs in the UAE and that the incidence of α-thal in the indigenous population is one of the highest in the world. Our clinical data suggest that Hb H disease in the UAE has, in general, a mild to moderate phenotypic presentation.


Article / Publication Details

First-Page Preview
Abstract of Original Paper

Published online: August 05, 1998
Issue release date: July 1998

Number of Print Pages: 5
Number of Figures: 0
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.