【先天性血小板出血性营养不良病史】。

Comptes rendus de l'Academie des sciences. Serie III, Sciences de la vie Pub Date : 1996-08-01

J Bernard

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引用次数: 0

摘要

这篇关于Bernard-Soulier综合征的历史回顾涉及:(1)疾病的第一次描述;(2) 1948年至1982年的主要研究数据，该研究发现了导致功能障碍(血小板粘附血管内皮下层的缺陷)的分子异常(血小板糖蛋白Ib缺乏或定性异常)，这些功能障碍现在被定义为疾病。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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[History of congenital thrombocytic hemorrhagic dystrophy].

This historical review of the Bernard-Soulier syndrome relates: (1) the first description of the disease; (2) the main data of the research from 1948 to 1982 which led to the discovery of the molecular abnormality (lack or qualitative abnormality of the platelet glycoprotein Ib) responsible for the functional disorder (defective platelet adhesion to the vascular subendothelium) which nowadays defines the disorder.

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Comptes rendus de l'Academie des sciences. Serie III, Sciences de la vie

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