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01-03-2012 | Editorial

Primary Biliary Cirrhosis: Bad Genes, Bad Luck

Authors: Pietro Invernizzi, M. Eric Gershwin

Published in: Digestive Diseases and Sciences | Issue 3/2012

Excerpt

Primary biliary cirrhosis (PBC) is considered a model for autoimmune disease based upon its hallmark autoantibodies serologic response, the focused target destruction of biliary epithelial cells (BEC), and the clinical homogeneity among patients [1]. PBC is also overwhelmingly a disease of middle-aged and older females, being virtually absent in the pediatric or adolescent population. True pediatric PBC is indeed highly rare, and likely represents an etiopathogenesis that is extremely atypical compared to the classical PBC in adults. PBC also has one of the latest ages of onset out of all autoimmune diseases [2]. The etiology remains enigmatic, although various genetic and environmental factors have been implicated in the disease development and progression, many of which also provide insight as to why females are more prone to PBC [3, 4]. …

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Title: Primary Biliary Cirrhosis: Bad Genes, Bad Luck
Authors: Pietro Invernizzi
M. Eric Gershwin
Publication date: 01-03-2012
Publisher: Springer US
Published in: Digestive Diseases and Sciences / Issue 3/2012
Print ISSN: 0163-2116
Electronic ISSN: 1573-2568
DOI: https://doi.org/10.1007/s10620-011-1993-3

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