论文部分内容阅读
本文报道8例遗传性因子Ⅰ、Ⅶ、X 缺乏症的家族调查、临床出血特点、实验室检查结果和治疗情况。所有病例均系常染色体隐性遗传,纯合子型者有严重出血倾向,杂合子型者出血轻微或不出血,但凝血酶原时间均明显延长。延长的凝血酶原时间可被正常血浆纠正者为因子Ⅰ缺乏症,被正常血清纠正且蝰蛇毒时间正常者为因子Ⅶ缺乏症,被正常血清纠正且蝰蛇毒时间延长者为因子 X 缺乏症。文中还对出血与遗传的关系、诊断和鉴别诊断以及治疗原则作了讨论。
This article reports 8 cases of hereditary factors Ⅰ, Ⅶ, X deficiency familial investigation, clinical bleeding characteristics, laboratory test results and treatment. All cases were autosomal recessive, homozygotes severe bleeding tendency, heterozygous minor bleeding or no bleeding, but the prothrombin time were significantly longer. Prolonged prothrombin time can be normal plasma correlates for factor I deficiency, is corrected by normal serum and snake venom is normal for the time due to factor VII deficiency, corrected by normal serum and snake venom for a prolonged time as factor X deficiency. The article also on bleeding and genetic relationship, diagnosis and differential diagnosis and treatment principles are discussed.