Journal
SEMINARS IN PEDIATRIC SURGERY
Volume 21, Issue 3, Pages 192-200Publisher
ELSEVIER SCIENCE INC
DOI: 10.1053/j.sempedsurg.2012.05.003
Keywords
Biliary atresia; Adaptive immunity; Innate immunity; Neonatal cholestasis
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Funding
- NIDDK NIH HHS [1 R01 DK078195, R01 DK078195] Funding Source: Medline
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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. (C) 2012 Elsevier Inc. All rights reserved.
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