摘要
<正>遗传性凝血因子Ⅶ缺陷症(hereditary factor Ⅶ deficiency)是一种常染色体隐性遗传病,系凝血因子Ⅶ(FⅦ)基因突变引起血浆中FⅦ功能发生障碍和(或)含量减少,影响外源性凝血途径的起始阶段从而导致临床出血症状的疾病,男女均可罹患~([1])。其实验室检查特点:凝血酶原时间延长,活化部分凝血活酶时间正常,FⅦ活性测定减低。该病由Alexander~([2])在1951年首次报道,约18%的患者与近亲婚配有关,发病率约为1/50
引文
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