Immune-mediated cholangiopathies in children: the need to better understand the pathophysiology for finding the future possible treatment targets
Alina Grama,
Alexandra Mititelu,
Claudia Sîrbe
et al.
Abstract:Cholangiopathies are defined as focal or extensive damage of the bile ducts. According to the pathogenetic mechanism, it may be immune-mediated or due to genetic, infectious, toxic, vascular, and obstructive causes. Their chronic evolution is characterized by inflammation, obstruction of bile flow, cholangiocyte proliferation, and progression toward fibrosis and cirrhosis. Immune-mediated cholangiopathies comprise primary sclerosing cholangitis (PSC), autoimmune cholangitis and IgG4-associated cholangitis in a… Show more
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