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Semin Pediatr Surg. 2012 Aug;21(3):192-200. doi: 10.1053/j.sempedsurg.2012.05.003.

Biliary atresia: cellular dynamics and immune dysregulation.

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  • 1Section of Pediatric Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO 80045, USA.

Abstract

The cause of biliary atresia is unknown; in the past few decades, the majority of investigations related to its pathogenesis have centered on viral infections and immunity. The acquired or perinatal form of biliary atresia entails a progressive inflammatory injury of bile ducts, leading to fibrosis and obliteration of both the extrahepatic and intrahepatic bile ducts. Theories of pathogenesis include viral infection, chronic inflammatory or autoimmune-mediated bile duct injury, and abnormalities in bile duct development. This review will focus solely on human studies pertaining to a potential viral trigger of bile duct injury at diagnosis and provide insight into the interplay of the innate and adaptive immune responses in the pathogenesis of disease.

Copyright © 2012 Elsevier Inc. All rights reserved.

PMID:
22800972
[PubMed - indexed for MEDLINE]
PMCID:
PMC3399127
Free PMC Article
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