HbE β-Thalassaemia in Malaysia: revisited

HbE β – thalassaemia is a public health problem in Malaysia and the most common type of thalassaemia seen in the Malays. It shows considerable diverse phenotypes. Complete molecular analysis to identify primary/ secondary alleles of thalassaemia and gene modifiers are arbitrary predictors of possib...

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Main Author: George, Elizabeth
Format: Article
Language:English
Published: Omics Publishing Group 2013
Online Access:http://psasir.upm.edu.my/id/eprint/29673/1/HbE%20%CE%B2.pdf
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author George, Elizabeth
author_facet George, Elizabeth
author_sort George, Elizabeth
collection UPM
description HbE β – thalassaemia is a public health problem in Malaysia and the most common type of thalassaemia seen in the Malays. It shows considerable diverse phenotypes. Complete molecular analysis to identify primary/ secondary alleles of thalassaemia and gene modifiers are arbitrary predictors of possible outcome of disease. Early diagnosis is important. Patients need to be classified as minor, moderate (TI) and severe. Clinical diagnosis requires careful observations over a period of time with good record keeping of growth, sexual maturation and quality of life. Patients with haemoglobin (Hb) levels less than 7 gm/dl should be treated as transfusion dependent β-thalassaemia major to prevent complications that occur progressively with advancing age. Hb levels less than 7 gm/dl show patients are destined to be short, have splenomegaly and skeletal abnormalities. Pre transfusion mean Hb levels kept between 9-10 gm/dl by transfusion will suppress bone marrow activity and decrease iron absorption through gastrointestinal tract.
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spelling upm.eprints-296732015-10-27T02:37:51Z http://psasir.upm.edu.my/id/eprint/29673/ HbE β-Thalassaemia in Malaysia: revisited George, Elizabeth HbE β – thalassaemia is a public health problem in Malaysia and the most common type of thalassaemia seen in the Malays. It shows considerable diverse phenotypes. Complete molecular analysis to identify primary/ secondary alleles of thalassaemia and gene modifiers are arbitrary predictors of possible outcome of disease. Early diagnosis is important. Patients need to be classified as minor, moderate (TI) and severe. Clinical diagnosis requires careful observations over a period of time with good record keeping of growth, sexual maturation and quality of life. Patients with haemoglobin (Hb) levels less than 7 gm/dl should be treated as transfusion dependent β-thalassaemia major to prevent complications that occur progressively with advancing age. Hb levels less than 7 gm/dl show patients are destined to be short, have splenomegaly and skeletal abnormalities. Pre transfusion mean Hb levels kept between 9-10 gm/dl by transfusion will suppress bone marrow activity and decrease iron absorption through gastrointestinal tract. Omics Publishing Group 2013 Article PeerReviewed application/pdf en http://psasir.upm.edu.my/id/eprint/29673/1/HbE%20%CE%B2.pdf George, Elizabeth (2013) HbE β-Thalassaemia in Malaysia: revisited. Journal of Hematology & Thromboembolic Diseases, 1 (1). pp. 1-3. ISSN 2329-8790 http://esciencecentral.org/journals/hbe-thalassaemia-in-malaysia-revisited-2329-8790.1000101.php?aid=11495 10.4172/2329-8790.1000101
spellingShingle George, Elizabeth
HbE β-Thalassaemia in Malaysia: revisited
title HbE β-Thalassaemia in Malaysia: revisited
title_full HbE β-Thalassaemia in Malaysia: revisited
title_fullStr HbE β-Thalassaemia in Malaysia: revisited
title_full_unstemmed HbE β-Thalassaemia in Malaysia: revisited
title_short HbE β-Thalassaemia in Malaysia: revisited
title_sort hbe β thalassaemia in malaysia revisited
url http://psasir.upm.edu.my/id/eprint/29673/1/HbE%20%CE%B2.pdf
work_keys_str_mv AT georgeelizabeth hbebthalassaemiainmalaysiarevisited