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Haemoglobin E beta thalassaemia in Sri Lanka.
Lancet 2005 Oct 22-28; 366(9495):1467-70Lct

Abstract

Haemoglobin E beta thalassaemia is the commonest form of severe thalassaemia in many Asian countries, but little is known about its natural history, the reasons for clinical diversity, or its management. We studied 109 Sri Lankan patients with the disorder over 5 years. 25 patients were not receiving transfusion; transfusion was stopped with no deleterious effect in a further 37. We identified several genetic and environmental factors that might contribute to the phenotypic diversity of the disorder, including modifiers of haemoglobin F production, malaria, and age-related changes in adaptive function. Our findings suggest that haemoglobin E beta thalassaemia can be managed without transfusion in many patients, even with low haemoglobin levels. Age-related changes in the pattern of adaptation to anaemia suggest that different and more cost-effective approaches to management should be explored.

Authors+Show Affiliations

National Thalassaemia Centre, General Hospital, Kurunegala, Sri Lanka.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

16243092

Citation

Premawardhena, A, et al. "Haemoglobin E Beta Thalassaemia in Sri Lanka." Lancet (London, England), vol. 366, no. 9495, 2005, pp. 1467-70.
Premawardhena A, Fisher CA, Olivieri NF, et al. Haemoglobin E beta thalassaemia in Sri Lanka. Lancet. 2005;366(9495):1467-70.
Premawardhena, A., Fisher, C. A., Olivieri, N. F., de Silva, S., Arambepola, M., Perera, W., ... Weatherall, D. J. (2005). Haemoglobin E beta thalassaemia in Sri Lanka. Lancet (London, England), 366(9495), pp. 1467-70.
Premawardhena A, et al. Haemoglobin E Beta Thalassaemia in Sri Lanka. Lancet. 2005;366(9495):1467-70. PubMed PMID: 16243092.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Haemoglobin E beta thalassaemia in Sri Lanka. AU - Premawardhena,A, AU - Fisher,C A, AU - Olivieri,N F, AU - de Silva,S, AU - Arambepola,M, AU - Perera,W, AU - O'Donnell,A, AU - Peto,T E A, AU - Viprakasit,V, AU - Merson,L, AU - Muraca,G, AU - Weatherall,D J, PY - 2005/10/26/pubmed PY - 2005/11/9/medline PY - 2005/10/26/entrez SP - 1467 EP - 70 JF - Lancet (London, England) JO - Lancet VL - 366 IS - 9495 N2 - Haemoglobin E beta thalassaemia is the commonest form of severe thalassaemia in many Asian countries, but little is known about its natural history, the reasons for clinical diversity, or its management. We studied 109 Sri Lankan patients with the disorder over 5 years. 25 patients were not receiving transfusion; transfusion was stopped with no deleterious effect in a further 37. We identified several genetic and environmental factors that might contribute to the phenotypic diversity of the disorder, including modifiers of haemoglobin F production, malaria, and age-related changes in adaptive function. Our findings suggest that haemoglobin E beta thalassaemia can be managed without transfusion in many patients, even with low haemoglobin levels. Age-related changes in the pattern of adaptation to anaemia suggest that different and more cost-effective approaches to management should be explored. SN - 1474-547X UR - https://www.unboundmedicine.com/medline/citation/16243092/Haemoglobin_E_beta_thalassaemia_in_Sri_Lanka_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0140-6736(05)67396-5 DB - PRIME DP - Unbound Medicine ER -