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.

引用

页码：33 / 39

页数：7

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