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Hepatic involvement in hereditary hemorrhagic telangiectasia: An unusual indication for liver transplantation
Author(s) -
Hillert Christian,
Broering Dieter C.,
Gundlach Matthias,
Knoefel Wolfram T.,
Izbicki Jakob R.,
Rogiers Xavier
Publication year - 2001
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1053/jlts.2001.21313
Subject(s) - medicine , liver transplantation , telangiectasia , liver disease , decompensation , transplantation , liver function , disease , cirrhosis , gastroenterology , surgery
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.