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约旦的遗传性出血综合征。

Inherited bleeding syndromes in Jordan.

作者信息

Alsabti E A, Hammadi M

出版信息

Acta Haematol. 1979;61(1):47-51. doi: 10.1159/000207625.

Abstract

This paper presents data on the occurrence and pattern of inherited bleeding syndromes (IBS) in Jordan, a hitherto unexplored problem. In 1978, during the first 12 months of a prospective study at a major medical center, 91 patients from 51 families were diagnosed as having IBS. All patients were referred because of moderate-to-severe bleeding diatheses; they included 52 hemophiliacs, 27 patients with von Willebrand's disease, 4 with hemophilia B (IX-deficit), 2 with afibrinogenemia, 1 with prothrombin deficiency, and 4 were thought to have platelet dysfunction. The clinical and laboratory features of the patients observed in Jordan do not seem to be significantly different from those of patients in Western Europe or North America.

摘要

本文介绍了约旦遗传性出血性疾病(IBS)的发生情况和模式,这是一个此前未被探索过的问题。1978年,在一家大型医疗中心进行的前瞻性研究的头12个月里,来自51个家庭的91名患者被诊断患有IBS。所有患者均因中度至重度出血素质而被转诊;其中包括52名血友病患者、27名血管性血友病患者、4名B型血友病(IX缺乏症)患者、2名无纤维蛋白原血症患者、1名凝血酶原缺乏症患者,还有4名被认为存在血小板功能障碍。在约旦观察到的患者的临床和实验室特征似乎与西欧或北美的患者没有显著差异。

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