Liver Unit, Pomeranian Medical University, Al. Powstancow Wlkp. 72, 70-111, Szczecin, Poland.
Front Biosci (Landmark Ed). 2012 Jan 1;17(3):959-69. doi: 10.2741/3966.
Primary biliary cirrhosis and primary sclerosing cholangitis are two most frequently encountered cholestatic condition in adults. Liver transplantation is an excellent option in patients who progress to end-stage liver disease. In these patients typical indications for liver transplantation are no different than in other conditions requiring transplantation. Liver transplantation however might be also suggested for specific indications even in patients with otherwise well preserved liver function. 5-year survival after liver transplantation in primary biliary cirrhosis was reported to be between 78 and 87%. Survival rates after liver transplantation in primary sclerosing cholangitis are considered favorable when compared to many other indications for this procedure. Nevertheless, in both primary biliary cirrhosis and primary sclerosing cholangitis recurrence of the disease after liver transplantation can be observed. Cystic fibrosis, biliary atresia, Alagille syndrome and progressive familiar intrahepatic cholestasis are cholestatic conditions in children. Liver transplantation represents an effective treatment in majority of cases fulfilling the criteria for this procedure.
原发性胆汁性肝硬化和原发性硬化性胆管炎是成人中最常见的两种胆汁淤积性疾病。对于进展为终末期肝病的患者，肝移植是一种极好的选择。在这些患者中，肝移植的典型适应证与其他需要移植的疾病没有区别。然而，即使在肝功能良好的患者中，也可能因特定适应证而建议进行肝移植。据报道，原发性胆汁性肝硬化患者肝移植后的 5 年生存率在 78%至 87%之间。与许多其他适应证相比，原发性硬化性胆管炎患者肝移植后的生存率被认为是有利的。然而，在原发性胆汁性肝硬化和原发性硬化性胆管炎中，肝移植后疾病的复发是可以观察到的。囊性纤维化、胆道闭锁、Alagille 综合征和进行性家族性肝内胆汁淤积症是儿童期的胆汁淤积性疾病。在大多数符合该手术标准的病例中，肝移植是一种有效的治疗方法。
