title: Improvement of ischemic cholangiopathy in three patients with hereditary hemorrhagic telangiectasia following treatment with bevacizumab.
abstract: The ischemic biliary phenotype of hereditary hemorrhagic telangiectasia (HHT) is rare but distinct, with progressive biliary tree ischemia usually resulting in an irreversible secondary sclerosing cholangiopathy. When clinically severe, liver transplant is often indicated. We report three patients with marked HHT associated biliary disease, in whom prolonged anti-vascular endothelial growth factor therapy (bevacizumab) notably reversed imaging evidence of biliary disease and clinically obviated need for liver transplantation during the first year of follow-up.
authors: Department of Diagnostic Imaging, St. Michael's Hospital, 30 Bond Street, Toronto, ON, Canada M5B 1W8.
authors: Paraskevi A
authors: PA
authors: Vlachou
authors: Errol
authors: E
authors: Colak
authors: Alexander
authors: A
authors: Koculym
authors: Anish
authors: A
authors: Kirpalani
authors: Tae Kyoung
authors: TK
authors: Kim
authors: Gideon M
authors: GM
authors: Hirschfield
authors: Marie E
authors: ME
authors: Faughnan
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D000068258:Bevacizumab; D001649:Bile Duct Diseases / Q000209:etiology* / Q000628:therapy*; D001653:Bile Ducts, Intrahepatic / Q000098:blood supply; D005260:Female; D006801:Humans; D007511:Ischemia / Q000209:etiology* / Q000628:therapy*; D016031:Liver Transplantation; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000628:therapy*; D016896:Treatment Outcome; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D000068258:Bevacizumab