Al-Mondhiry H A
Thromb Haemost. 1977 Jun 30;37(3):549-55.
This paper presents data on the occurence and pattern of inherited bleeding syndromes (IBS) in Iraq, a hitherto unexplored problem. During the first fourteen months of a prospective on-going study at a major university center, 116 patients from 62 families were diagnosed as having IBS. All patients were referred because of moderate to severe bleeding diatheses. They included 62 haemophiliacs 32 patients with von Willebrand's disease (VWD), 9 with Christmas disease (CD), 6 with afibrinogenemia, 1 with prothrombin deficiency, and 6 were thought to have platelet dysfunction. 32 other bleeders (16 hemophiliacs, 14 VWD, and 2 CD) were also recognized among the pedigrees studied but were not available for full investigations. The clinical and laboratory features of the patients observed in Iraq do not seem to be significantly different from those of patients in Western Europe or North America. Although the absolute incidence and relative distribution of these disorders in the entire population cannot yet be determined, the rate of occurence per segment population is likely to be high, most likely due to the high rate of consanguinity and large number of births per family, phenomena still prevalent in this country.
本文介绍了伊拉克遗传性出血性疾病（IBS）的发生情况和模式，这是一个此前未被探索的问题。在一所主要大学中心进行的一项前瞻性持续研究的头14个月里，来自62个家庭的116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62名血友病患者、32名血管性血友病（VWD）患者、9名克里斯马斯病（CD）患者、6名无纤维蛋白原血症患者、1名凝血酶原缺乏症患者，还有6名被认为存在血小板功能障碍。在研究的家系中还识别出另外32名出血者（16名血友病患者、14名VWD患者和2名CD患者），但无法对他们进行全面调查。在伊拉克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。尽管这些疾病在整个人口中的绝对发病率和相对分布尚无法确定，但按部分人群计算的发生率可能很高，这很可能是由于该国近亲结婚率高且每个家庭生育子女数量多，这些现象在该国仍然普遍存在。
