Please use this identifier to cite or link to this item: http://repository.kln.ac.lk/handle/123456789/1985
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dc.contributor.authorAllen, A.en_US
dc.contributor.authorFisher, C.en_US
dc.contributor.authorPremawardhena, A.en_US
dc.contributor.authorPeto, T.en_US
dc.contributor.authorAllen, S.J.en_US
dc.contributor.authorArambepola, M.en_US
dc.contributor.authorThayalsuthan, V.en_US
dc.contributor.authorOlivieri, N.en_US
dc.contributor.authorWeatherall, D.en_US
dc.date.accessioned2014-10-29T09:28:40Z
dc.date.available2014-10-29T09:28:40Z
dc.date.issued2010en_US
dc.identifier.citationBlood. 2010; 116(24): 5368-70. [ Erratum in: Blood. 2011 22;118(26):6994-5]en_US
dc.identifier.issn0006-4971 (Print)en_US
dc.identifier.issn1528-0020 (Electronic)en_US
dc.identifier.urihttp://repository.kln.ac.lk/handle/123456789/1985
dc.descriptionIndexed in MEDLINE
dc.description.abstractHemoglobin E beta thalassemia is the commonest form of severe thalassemia in many Asian countries. Its remarkably variable clinical phenotype presents a major challenge to determining its most appropriate management. In particular, it is not clear why some patients with this condition can develop and function well at very low hemoglobin levels. Here, we demonstrate that patients with hemoglobin E beta thalassemia have a significant decrease in the oxygen affinity of their hemoglobin, that is an increased P(50) value, in response to anemia. This may in part reflect the lower level of hemoglobin F in this condition compared with other forms of beta thalassemia intermedia. The ability to right-shift the oxygen dissociation curve was retained across the spectrum of mild and severe phenotypes, despite the significantly higher levels of hemoglobin F in the former, suggesting that efforts directed at producing a modest increase in the level of hemoglobin F in symptomatic patients with this disease should be of therapeutic value.en_US
dc.publisherAmerican Society of Hematologyen_US
dc.subjectbeta-Thalassemiaen_US
dc.subjectAnemia-blooden_US
dc.subjectHemoglobin E-metabolismen_US
dc.subjectOxygen-metabolismen_US
dc.subjectbeta-Thalassemia-blooden_US
dc.subjectbeta-Thalassemia-complicationsen_US
dc.subjectFetal Hemoglobin-analysisen_US
dc.titleAdaptation to anemia in hemoglobin E-beta thalassemiaen_US
dc.typeArticleen_US
dc.identifier.departmentMedicineen_US
dc.creator.corporateauthorAmerican Society of Hematologyen_US
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