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Liver failure caused by hepatic angiodysplasia in hereditary hemorrhagic telangiectasia

Authors
Journal
The American Journal of Gastroenterology
0002-9270
Publisher
Nature Publishing Group
Publication Date
Volume
93
Issue
3
Identifiers
DOI: 10.1016/s0002-9270(98)80052-x
Disciplines
  • Medicine

Abstract

Hereditary hemorrhagic telangiectasia is a systemic vascular disease with autosomal dominant inheritance that results in telangiectasia, arteriovenous malformations, and hemangiomas. The liver is one of the organs commonly affected in hereditary hemorrhagic telangiectasia, and hepatic lesions consist of angiodysplasia and fibrosis. A patient with hereditary hemorrhagic telangiectasia and significant impairment of synthetic liver function is reported. Dynamic computed tomography revealed marked enlargement of the common hepatic and intrahepatic arteries, heterogeneous parenchymography, and early opacification of the hepatic veins consistent with telangiectasias and arteriovenous shunting. Overall, the liver was predominantly occupied by vascular structures and scarce residual hepatic parenchyma. Other causes of liver dysfunction, such as viral hepatitis and alcohol abuse, were excluded. In general, hepatic fibrovascular dysplasia seen in hereditary hemorrhagic telangiectasia usually results in only mild liver dysfunction; however, this case shows that hepatic involvement may rarely result in hepatic failure.

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