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Hemoglobin Bart's Levels in Umbilical Cord Blood: Failure as a Method for Distinguishing Mild from Severeα-Thalassemia Trait in the Chinese

 

作者: ToddD.,   ChanT. K.,  

 

期刊: Hemoglobin  (Taylor Available online 1978)
卷期: Volume 2, issue 4  

页码: 389-392

 

ISSN:0363-0269

 

年代: 1978

 

DOI:10.3109/03630267809005348

 

出版商: Taylor&Francis

 

数据来源: Taylor

 

摘要:

α-Thalassemia is associated with a defect in the production of theα-globin chains of hemoglobin. There are four common varieties in decreasing order of severity: the hemoglobin (Hb) Bart's (γ4) hydrops fetalis syndrome, hemoglobin H disease,“severe”heterozygousα-thalassemia orα-thal-1 trait and“mild”heterozygousα-thalassemia orα-thal-2 trait (1) and there is now evidence that these may be the result of deletion of 4, 3, 2, and 1 respectively of theα-globin structural genes (2, 3, 4). As a result of defectiveα-globin chain production, the umbilical cord blood of infants with Hb Bart's hydrops fetalis contains from 80 to 90 per cent Hb Bart's (1) and those with Hb H disease, about 26 per cent (5). In newborn infants withα-thalassemia trait, lesser amounts of Hb Bart's have been found (5, 6, 7, 8). Observations in Thailand suggest that they fall into two groups, those with about 5 per cent Hb Bart's and others with from 1-2 per cent (9). The former were designated to haveα-thal-1 trait and the latter theα-thal-2 trait. However, there is uncertainty as to whether a clear-cut division between these two groups of infants can be made on this basis (1) and in the present study we have been unable to find a bi-modal distribution of Hb Bart's levels in the cord blood of Chinese infants withα-thalassemia trait.

 

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