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
收藏
页码：33 / 39
页数：7
相关论文
共 13 条
[1]   Thalassaemia in Sri Lanka: implications for the future health burden of Asian populations [J].
de Silva, S ;
Fisher, CA ;
Premawardhena, A ;
Lamabadusuriya, SP ;
Peto, TEA ;
Perera, G ;
Old, JM ;
Clegg, JB ;
Olivieri, NF ;
Weatherall, DJ .
LANCET, 2000, 355 (9206) :786-791
[2]   The molecular basis for the thalassaemias in Sri Lanka [J].
Fisher, CA ;
Premawardhena, A ;
de Silva, S ;
Perera, G ;
Rajapaksa, S ;
Olivieri, NA ;
Old, JM ;
Weatherall, DJ .
BRITISH JOURNAL OF HAEMATOLOGY, 2003, 121 (04) :662-671
[3]   Hemoglobinopathies in Southeast Asia: Molecular biology and clinical medicine [J].
Fucharoen, S ;
Winichagoon, P .
HEMOGLOBIN, 1997, 21 (04) :299-319
[4]   Genetic determinants of jaundice and gallstones in haemoglobin E β thalassaemia [J].
Premawardhena, A ;
Fisher, CA ;
Fathiu, F ;
de Silva, S ;
Perera, W ;
Peto, TEA ;
Olivieri, NF ;
Weatherall, DJ .
LANCET, 2001, 357 (9272) :1945-1946
[5]  
PREMAWARDHENA A, 2005, IN PRESS LANCET, V366
[6]   HIGH-SENSITIVITY OF DETECTION OF HUMAN MALARIA PARASITES BY THE USE OF NESTED POLYMERASE CHAIN-REACTION [J].
SNOUNOU, G ;
VIRIYAKOSOL, S ;
ZHU, XP ;
JARRA, W ;
PINHEIRO, L ;
DOROSARIO, VE ;
THAITHONG, S ;
BROWN, KN .
MOLECULAR AND BIOCHEMICAL PARASITOLOGY, 1993, 61 (02) :315-320
[7]   Infant parasite rates and immunoglobulin in seroprevalence as a measure of exposure to Plasmodium falciparum during a randomized controlled trial of insecticide-treated bed nets on the Kenyan coast [J].
Snow, RW ;
Molyneux, CS ;
Warn, PA ;
Omumbo, J ;
Nevill, CG ;
Gupta, S ;
Marsh, K .
AMERICAN JOURNAL OF TROPICAL MEDICINE AND HYGIENE, 1996, 55 (02) :144-149
[8]  
Sonakul D, 1992, Southeast Asian J Trop Med Public Health, V23 Suppl 2, P25
[9]  
WASI P, 1982, BIRTH DEFECTS-ORIG, V18, P213
[10]   ALPHA- AND BETA-THALASSEMIA IN THAILAND [J].
WASI, P ;
NANAKORN, S ;
POOTRAKUL, SN ;
SOOKANEK, M ;
DISTHASONGCHAN, P ;
PORNPATKUL, M ;
PANICH, V .
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES, 1969, 165 (A1) :60-+
12