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[杂合子β地中海贫血(作者译)]

[Heterocygous beta thalassaemia (author's transl)].

作者信息

Méndez Aparicio F

出版信息

An Esp Pediatr. 1978 Aug-Sep;11(8-9):609-16.

PMID:717922
Abstract

Two girls with an heterocigotic beta-thalassemy are presented in this study. Case 1 has an hypochromic and microtic anaemia with an enormous splenomegaly, increased osmotic resistence of red blood cells in salted solution and increase of A2 hemoglobin. This situation is associated with an increase of the glucolitic intraerythrocitic enzimes. Case 2 showed increase of A2 hemoglobine, but this anomaly was associated with decrease of intraerythrocitic enzimatic rate. First clinical signs of erythrocitic disturbances was an acute hemolytic crisis developed by the supply of the sulphometoxipiridacine. The erythroquinetic study showed a decrease of the average life of the red blood cells in both patients.

摘要

本研究报告了两名患有杂合子β地中海贫血的女孩。病例1有低色素性小红细胞性贫血,伴有巨大脾肿大,盐溶液中红细胞的渗透抵抗力增加,以及A2血红蛋白增加。这种情况与红细胞内糖酵解酶增加有关。病例2显示A2血红蛋白增加,但这种异常与红细胞内酶活性降低有关。红细胞紊乱的最初临床症状是在使用磺甲氧基吡拉西啶后发生的急性溶血危机。红细胞动力学研究显示,两名患者的红细胞平均寿命均缩短。

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