Martinić P, Brusić B
Bilt Hematol Transfuz. 1979;7(1):73-6.
Congenital deficit of Factor VII is a rare deficit perceived within women and men. Clinicaly it is manifested with mild hemoragical diatesa and of laboratory tests: prolonged on stage prothrombin time, reduced activity of Factor VII and normal APTT. In this article we describe the family in which we have found two cases of congenital deficit of Factor VII, biochemical characteristics, differential laboratory diagnosis and correction of deficit in the case of bleeding.
先天性因子VII缺乏是一种在男性和女性中均有发现的罕见病症。临床上表现为轻度出血倾向,实验室检查可见:凝血酶原时间延长、因子VII活性降低以及活化部分凝血活酶时间(APTT)正常。在本文中,我们描述了一个发现两例先天性因子VII缺乏病例的家族,介绍了其生化特征、实验室鉴别诊断以及出血时缺乏症的纠正方法。