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Symptomatic hepatic involvement is a rare complication of hereditary hemorrhagic telangiectasia and most commonly consists of fibrosis or cirrhosis. We describe a 33-year-old woman in whom multiple hepatic arteriovenous malformations led to high output cardiac failure and liver failure due to biliary necrosis with refractory biliary sepsis, requiring orthotopic liver transplantation. Hepatic arteriovenous malformations were the first manifestation of the disease and a similar asymptomatic hepatic tumour was subsequently detected in her 60-year-old father who also had the classical cutaneous stigmata of the syndrome. Unrecognised genetic factors may determine the clinical spectrum of hereditary haemorrhagic telangiectasia including the hepatic manifestations.

Original publication

DOI

10.1016/0168-8278(95)80455-2

Type

Journal article

Journal

J Hepatol

Publication Date

05/1995

Volume

22

Pages

586 - 590

Keywords

Adult, Arteriovenous Malformations, Female, Humans, Liver, Liver Failure, Liver Transplantation, Pedigree, Sepsis, Telangiectasia, Hereditary Hemorrhagic