Rifai K, Manns M P, Bahr M J
Abt. Gastroenterologie, Hepatologie und Endokrinologie, Medizinische Hochschule Hannover.
Z Gastroenterol. 2004 Aug;42(8):749-65. doi: 10.1055/s-2004-813182.
The clinical presentation of metabolic liver disease is highly variable, covering acute liver failure, liver cirrhosis, hepatic cancer and various extrahepatic manifestations. Both natural course and prognosis after liver transplantation are substantially influenced by extrahepatic manifestations. In many types of metabolic liver disease, timely diagnosis allows for successful medical treatment. However, progressive liver failure and severe extrahepatic damage can be the indication for liver transplantation. In general, standard transplantation criteria also apply for metabolic liver disease. They have to be modified by disease-specific criteria, and extrahepatic damage may necessitate multiorgan transplantation. The overall prognosis after liver transplantation for metabolic liver disease is favorable. Furthermore, several metabolic defects are phenotypically cured by liver transplantation. Alternative treatments like hepatocyte transplantation or gene therapy are still in the experimental stage.
代谢性肝病的临床表现高度多变,涵盖急性肝衰竭、肝硬化、肝癌以及各种肝外表现。肝外表现对肝移植后的自然病程和预后均有显著影响。在许多类型的代谢性肝病中,及时诊断可实现成功的药物治疗。然而,进行性肝衰竭和严重的肝外损害可能成为肝移植的指征。一般而言,标准的移植标准也适用于代谢性肝病。但这些标准必须根据疾病特异性标准进行调整,并且肝外损害可能需要进行多器官移植。代谢性肝病肝移植后的总体预后良好。此外,肝移植可从表型上治愈几种代谢缺陷。肝细胞移植或基因治疗等替代疗法仍处于实验阶段。