Liver transplantation for hepatic arteriovenous malformation in hereditary haemorrhagic telangiectasia.

Abstract:

: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.

journal_name

J Hepatol

journal_title

Journal of hepatology

authors

Bauer T,Britton P,Lomas D,Wight DG,Friend PJ,Alexander GJ

doi

10.1016/0168-8278(95)80455-2

subject

Has Abstract

pub_date

1995-05-01 00:00:00

pages

586-90

issue

5

eissn

0168-8278

issn

1600-0641

pii

0168-8278(95)80455-2

journal_volume

22

pub_type

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