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.

journal_name

J Hepatol

journal_title

Journal of hepatology

authors

Vlachou PA,Colak E,Koculym A,Kirpalani A,Kim TK,Hirschfield GM,Faughnan ME

doi

10.1016/j.jhep.2013.02.006

subject

Has Abstract

pub_date

2013-07-01 00:00:00

pages

186-9

issue

1

eissn

0168-8278

issn

1600-0641

pii

S0168-8278(13)00128-1

journal_volume

59

pub_type

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