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[先天性难治性贫血和继发性铁沉着症的长期研究]

[Long-term study in congenital refractory anemia and secondary siderosis].

作者信息

Tuschy U, Schmidt U, Meinhold J, Wutke K

出版信息

Z Gesamte Inn Med. 1982 Apr 1;37(7):213-7.

PMID:7102009
Abstract

It is reported on the course of the disease in a female patient with congenital therapy-refractory anaemia, in whom a transfusion siderosis of high degree with functional disturbances of the myocardium and the liver, with endocrine disturbances (primary hypothyroidism and normogonadotropic hypogonadism) and with small growth became clinically manifest. A reduction of the iron overload by a continuous desferrioxamine therapy in form of long-term subcutaneous infusions over two years could be proved. Possibilities and problems of treatment and conclusions relevant to practice are described.

摘要

报告了一名患有先天性难治性贫血的女性患者的疾病病程,该患者出现了高度输血性铁沉着症,伴有心肌和肝脏功能障碍、内分泌紊乱(原发性甲状腺功能减退和正常促性腺激素性性腺功能减退)以及生长发育迟缓,并已临床表现出来。通过连续两年长期皮下输注去铁胺疗法可证明铁过载有所减轻。描述了治疗的可能性和问题以及与实践相关的结论。

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