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[先天性溶血性贫血]

[Congenital hemolytic anemias].

作者信息

Schwarzmeier J D, Moser K, Pohl A

出版信息

Acta Med Austriaca. 1979;6(5):169-74.

PMID:555211
Abstract

During the last years our knowledge of the structural composition of erythrocyte membranes has markedly increased. It is the aim of this review to discuss hereditary changes of the erythrocyte membrane of pathophysiological significance on the basis of these recent biochemical findings. The report particularly deals with structural proteins, such as spectrin, and its possible significance for corpuscular deformation (e. g. spherocytosis). In addition, the importance of hemoglobinopathies and hereditary enzymatic defects for the development of hemolysis is discussed.

摘要

在过去几年里,我们对红细胞膜结构组成的了解有了显著增加。本综述的目的是基于这些最新的生化发现,讨论具有病理生理意义的红细胞膜遗传性变化。该报告特别涉及结构蛋白,如血影蛋白,及其对红细胞变形(如球形红细胞增多症)的可能意义。此外,还讨论了血红蛋白病和遗传性酶缺陷对溶血发展的重要性。

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