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甲型和乙型血友病携带者的出血症状。

Bleeding symptoms in carriers of hemophilia A and B.

作者信息

Mauser Bunschoten E P, van Houwelingen J C, Sjamsoedin Visser E J, van Dijken P J, Kok A J, Sixma J J

机构信息

Van Creveld Clinic, Bilthoven, The Netherlands.

出版信息

Thromb Haemost. 1988 Jun 16;59(3):349-52.

PMID:2847347
Abstract

In order to investigate the bleeding tendency in clinically identified carriers of hemophilia, a self-administered questionnaire was held among 135 carriers of hemophilia A and B, 25 females with relatives with hemophilia and a matched group consisting of 60 females without relatives with hemophilia. Carriers of hemophilia appeared to suffer more often from bleeding than their relatives or the matched unrelated control group. A relation was seen between factor VIII:C or IX:C activity and the tendency to bleed. Obligatory carriers with normal factor VIII:C levels showed no bleeding tendency and were in this respect similar to a group of 25 females with relatives with hemophilia. This study shows that it is important to assay factor VIII:C or IX:C also in those women in whom the carrier status has already been established otherwise.

摘要

为了调查临床确诊的血友病携带者的出血倾向,我们对135名甲型和乙型血友病携带者、25名有血友病亲属的女性以及由60名无血友病亲属的女性组成的匹配组进行了一项自填式问卷调查。血友病携带者似乎比其亲属或匹配的非相关对照组更容易出血。观察到因子VIII:C或IX:C活性与出血倾向之间存在关联。因子VIII:C水平正常的 obligatory 携带者没有出血倾向,在这方面与一组有血友病亲属的25名女性相似。这项研究表明,对于那些已确定为携带者状态的女性,检测因子VIII:C或IX:C也很重要。 (注:“obligatory carriers”可能是“义务携带者”之类的特定术语,具体准确含义需结合专业背景进一步确定,这里暂按原样翻译)

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