Hillert C, Broering D C, Gundlach M, Knoefel W T, Izbicki J R, Rogiers X
Department of Hepatobiliary Surgery, University Hospital, Universitäts-Krankenhaus-Eppendorf, Martinistr. 52, D-20246 Hamburg, Germany.
Liver Transpl. 2001 Mar;7(3):266-8. doi: 10.1053/jlts.2001.21313.
Hereditary hemorrhagic telangiectasia (HHT) is a vascular disease with multiple organ manifestations. Severe hepatic involvement is an unusal indication for liver transplantation. We report on a 39-year-old woman diagnosed with HHT and decompensation of the disease during her second pregnancy. After delivery, hemobilia occurred, followed by severe therapy-resistant cholangitis and progressive liver dysfunction. Because of progressive loss of liver function, orthotopic liver transplantation needed to be performed. The various aspects of liver involvement in Osler's disease, diagnosis, and therapy are discussed.
遗传性出血性毛细血管扩张症（HHT）是一种具有多器官表现的血管疾病。严重的肝脏受累是肝移植的罕见指征。我们报告了一名39岁女性，她在第二次怀孕期间被诊断为HHT且病情失代偿。产后发生了胆道出血，随后出现严重的难治性胆管炎和进行性肝功能障碍。由于肝功能进行性丧失，需要进行原位肝移植。本文讨论了奥斯勒病肝脏受累的各个方面、诊断及治疗。
