Publication: The molecular basis of AE-bart's disease
Issued Date
1989-01-01
Resource Type
ISSN
03630269
Other identifier(s)
2-s2.0-0024549950
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Mahidol University
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SCOPUS
Bibliographic Citation
Hemoglobin. Vol.13, No.2 (1989), 117-124
Suggested Citation
V. Thonglairuam, P. Winichagoon, S. Fucharoen, P. Wasi The molecular basis of AE-bart's disease. Hemoglobin. Vol.13, No.2 (1989), 117-124. doi:10.3109/03630268908998061 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/15726
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Title
The molecular basis of AE-bart's disease
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Abstract
AE-Bart's disease is a thalassemia intermedia resulting from the interaction between αthalassemia and heterozygous Hb E. In this study we analyzed the αglobin genes of 25 patients designated as AE-Bart's disease by starch gel electrophoresis. Twenty-one cases had Hb Constant Spring in addition to Hbs E + A + Bart's, and the remaining four cases had only Hbs E + A + Bart's. DNA mapping revealed the αglobin genotype of αthalassemia-1/αthalas-semia-2 in four patients who had Hbs E + A + Bart's, whereas the α genotype of the remainder is αthalassemia-1/nondeletion αthalassemia. The nondeletion αthalassemia is Hb Constant Spring as indicated by starch gel electrophoresis. Hematologic data and hemoglobin analysis showed that Constant Spring-AE-Bart's disease is a more severe clinical syndrome than AE-Bart's disease. © 1989 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted.