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α‐Thalassemia and β‐thalassemia in a turkish family

 

作者: C. Altay,   B. Say,   S. Yetgin,   Titus H. J. Huisman,  

 

期刊: American Journal of Hematology  (WILEY Available online 1977)
卷期: Volume 2, issue 1  

页码: 1-15

 

ISSN:0361-8609

 

年代: 1977

 

DOI:10.1002/ajh.2830020102

 

出版商: Wiley Subscription Services, Inc., A Wiley Company

 

关键词: α‐thalassemia;β‐thalassemia;in vitro synthesis ratio;silent genes;interaction of thalassemia determinants

 

数据来源: WILEY

 

摘要:

AbstractA Turkish family is described in which three children have a clinical picture similar to that of thalassemia major, with typical red cell morphology and indices, and with about 10% Hb Bart's but without measurable amounts of Hb H. Hematological evaluation of six members of this family that included in vitro hemoglobin synthesis suggests that β‐ (or δβ‐) thalassemia, β‐silent thalassemia, and mild and severe α‐thalassemia genes are present in different combinations. The data indicate that β/α chain ratios in patients with more than one type of thalassemia should be evaluated in relationship to values obtained for several relatives even though some of the thalassemia determinants may be silent

 

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