Liver transplantation for haemophiliacs with hepatitis C cirrhosis.
Liver transplantation for haemophiliacs with hepatitis C cirrhosis.
复制标题
丙型肝炎肝硬化血友病患者的肝移植。
作者：
Michelle McCarthy;Edward Gane;Stephen P. Pereira;C. Tibbs;N. Heaton;M. Rela;H. Hambley;Roger Williams
背景：终末期丙型肝炎血友病患者的肝移植经验有限，当同时存在HIV感染时，尤其提出了困难的问题。目的：这是英国首次报道四例终末期丙型肝炎肝硬变血友病患者的手术替代疗法和初步结果。患者：2例患者为凝血因子VIII，1例为凝血因子IX，1例为凝血因子X缺乏症。一名患者也因因子替代感染了艾滋病毒，但没有艾滋病定义的疾病。方法：根据精确计算凝血因子需要量的公式，术中给患者输注凝血因子VIII、凝血因子IX或新鲜冰冻血浆。术前基础凝血检查包括凝血酶原时间、活化部分凝血活酶时间、纤维蛋白原浓度和凝血因子VIII、IX和X浓度。然后在术后12、24、48和72小时测定因子浓度。结果：所有患者术后72小时内凝血因子水平均维持在正常范围内(137、125、95、104IU/dl)，代表移植物的从头合成。手术期间和移植后即刻的输血需求并不比那些没有凝血障碍的患者更多。两名患者术后有出血发作，其中一例是致命的，发生在先前未治疗的硬膜下出血部位。在这两种情况下，出血都发生在凝血因子浓度正常的情况下。其余3名患者分别在移植后6个月、6个月和12个月，通过持续的因子浓度显著改善临床症状。1例患者有丙型肝炎复发后移植物功能障碍的证据，所有患者在聚合酶链式反应研究中都有丙型肝炎病毒血症复发的证据。结论：在血友病患者合并或不合并HIV感染的终末期肝病患者中，应考虑进行原位肝移植。他们的临床状况可能会通过原位肝移植得到极大的改善，血友病被治愈，而由于复发的丙型肝炎病毒感染，只有很小的风险导致严重的移植物功能障碍。
BACKGROUND: Experience of liver transplantation in haemophiliacs with end stage hepatitis C liver disease is limited and particularly difficult questions are raised when there is also HIV infection. AIMS: This is the first report in Great Britain to describe the operative replacement therapy and initial outcome in four haemophiliacs with end stage HCV cirrhosis. PATIENTS: Two patients had factor VIII, one had factor IX, and one had factor X deficiency. One patient had also contracted HIV infection from factor replacement but had no AIDS defining illnesses. METHODS: Intraoperatively patients were given either factor VIII infusions, factor IX bolus, or fresh frozen plasma, according to formulae devised to calculate exact clotting factor requirements. Baseline preoperative coagulation studies included prothrombin time, activated partial thromboplastin time, fibrinogen concentrations, and factor VIII, IX, and X concentrations. Factor concentrations were then assayed at 12, 24, 48, and 72 hours postoperatively. RESULTS: Postoperatively all patients had coagulation factor concentrations sustained within the normal ranges by 72 hours unsupported (137, 125, 95, 104 IU/dl), representing de novo synthesis by the graft. Transfusion requirements during the operative and immediate post-transplant period were no greater than those of patients without clotting disorders. Two patients had episodes of bleeding postoperatively, one of which was fatal, occurring at the site of a previous untreated subdural bleed. 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.
多次输血血友病患者丙型肝炎病毒感染的自然史：与人类免疫缺陷病毒共感染的影响。
DOI：
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发表时间：
1993
期刊：
Journal of acquired immune deficiency syndromes
影响因子：
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作者：
Eyster,ME;Diamondstone,LS;Lien,JM;Ehmann,WC;Quan,S;Goedert,JJ
通讯作者：
Goedert,JJ