Hemoglobin E-β-thalassemia:: Progress report from the international study group

被引：11
作者：
Premawardhena, A
De Silver, S
Arambepola, M
Olivieri, NF
Vichinsky, EP
Merson, L
Muraco, G
Allen, A
Fisher, C
Peto, T
Weatherall, DJ [1 ]
机构：
[1] Univ Oxford, John Radcliffe Hosp, Weatherall Inst Mol Med, Oxford OX3 9DS, England
[2] Univ Kelaniya, Dept Med, Kelaniya, Sri Lanka
[3] Lady Ridgeway Hosp Children, Colombo, Sri Lanka
[4] Gen Hosp, Natl Thalassaemia Ctr, Kurunegala, Sri Lanka
[5] Univ Hlth Network, Toronto, ON, Canada
[6] Childrens Hosp, Oakland, CA 94609 USA
[7] Univ Oxford, Weatherall Inst Mol Med, Oxford, England
来源：
COOLEY'S ANEMIA EIGHTH SYMPOSIUM | 2005年 / 1054卷
关键词：
thalassemia; hemoglobin E;
D O I：
10.1196/annals.1345.005
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
A long-term observational study of Hb E-beta-thalassemia in Sri Lanka is beginning to define some of the genetic and environmental factors that are responsible for its remarkable phenotypic variability. In this population there is a very small difference between the steady-state hemoglobin levels between the mild and severe phenotypes, and it has been possible to stop transfusion in many of those who have been on long-term treatment of this kind. These preliminary observations, made over the last 7 years, provide directions for future research into this increasingly important disease.
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页码：33 / 39
页数：7
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