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约旦的先天性出血性疾病

Congenital hemorrhagic disorders in Jordan.

作者信息

Awidi A S

出版信息

Thromb Haemost. 1984 Jul 29;51(3):331-3.

PMID:6333734
Abstract

The results of a three year prospective study of inherited bleeding syndromes in Jordan is presented. There were 112 patients from 64 families. Of these there were 42 patients with hemophilia A, 23 with Glanzmann's thrombasthenia, 22 with von Willebrand's disease, 11 with Christmas disease, 6 with hypofibrinogenemia, 3 with afibrinogenemia, 2 with factor XIII deficiency, 2 with storage pool disease and 1 with factor XI deficiency. The pattern of inherited bleeding syndromes in Jordan is different from that seen in Europe and U.S.A. in that Glanzmann's thrombasthenia is very common. High proportion of hemophiliacs were severe. Arthropathy was common. A significant number of bleeders had fatal hemorrhage. In a high proportion of patients, no family history of bleeding was found.

摘要

本文介绍了一项针对约旦遗传性出血综合征的为期三年的前瞻性研究结果。研究对象来自64个家庭,共112名患者。其中,42例为A型血友病患者,23例为Glanzmann血小板无力症患者,22例为血管性血友病患者,11例为克里斯马斯病患者,6例为低纤维蛋白原血症患者,3例为无纤维蛋白原血症患者,2例为因子XIII缺乏症患者,2例为储存池病患者,1例为因子XI缺乏症患者。约旦遗传性出血综合征的模式与欧洲和美国不同,Glanzmann血小板无力症非常常见。血友病患者中重度患者比例较高。关节病很常见。相当数量的出血者发生了致命性出血。在很大一部分患者中,未发现出血家族史。

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