文摘

背景:血友病患者肝移植的经验与结束阶段丙型肝炎肝病是有限的特别困难的问题,也有感染艾滋病毒。目的:这是第一个报告在英国来描述有效的替代疗法和初始结果血友病患者在四丙肝肝硬化晚期。病人:两个患者因素八世,第九人的因素,人的因素X不足。一个病人也感染了艾滋病病毒感染因素替代但没有定义疾病艾滋病。方法:参考患者要么VIII因子注入,因子IX丸,或新鲜冷冻血浆,根据公式设计计算确切的凝血因子的要求。基线术前凝固研究包括凝血酶原时间、活化部分血栓形成质时间、纤维蛋白原浓度,浓度和VIII因子,第九,X。因素浓度被化验12、24、48和72小时术后。结果:术后所有患者凝血因子浓度维持在正常范围,72小时不支持(137、125、95、104 IU / dl),代表从头合成的贪污。输血需求手术和直接移植后期间没有大于无凝血障碍的患者。两个病人的术后出血,其中一个是致命的,发生在前一个未经处理的硬膜下出血。 In both instances the bleeding occurred in the presence of normal concentrations of clotting factor. The remaining three patients are at 6, 6, and 12 months post-transplant and remarkably improved clinically with sustained factor concentrations. One patient has evidence of graft dysfunction from HCV recurrence and all have evidence of recurrent viraemia with HCV on polymerase chain reaction studies. CONCLUSIONS: Orthotopic liver transplantation should be considered in haemophiliac patients with end stage liver disease from hepatitis C infection with or without concomitant HIV infection. Their clinical condition is likely to be greatly improved by orthotopic liver transplantation and the haemophilia cured with only a small risk of severe graft dysfunction from recurrent HCV infection.