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Model for end‐stage liver disease (MELD) exception for hereditary hemorrhagic telangiectasia
Author(s) -
GarciaTsao Guadalupe,
Gish Robert G.,
Punch Jeffrey
Publication year - 2006
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 - 10.1002/lt.20978
Subject(s) - medicine , telangiectasia , telangiectases , liver transplantation , portal hypertension , cirrhosis , pathology , liver disease , portopulmonary hypertension , gastrointestinal bleeding , gastroenterology , transplantation
Hereditary hemorrhagic telangiectasia (HHT), also called Rendu-Osler-Weber syndrome, is a rare autoso- mal dominant disease characterized by arteriovenous malformations involving the skin, mucous membranes, lungs, brain, and gastrointestinal tract. The genetic or- igin of this disease appears to be a defect in transmem- brane proteins that are components of the receptor complex for transforming growth factor- (endoglin, ac- tivin receptor like-kinase 1). Hepatic involvement is fre- quent, but it is asymptomatic in most cases. HHT is characterized by diffuse vascular malformations throughout the liver that result in multiple types of shunting (hepatic artery to hepatic vein, hepatic artery to portal vein, portal vein to hepatic vein), with hepatic- artery-to-hepatic-vein shunting being the predominant type.