Background: Biliary atresia (BA) is the most common cause of obstructive jaundice in infants. Although the Kasai procedure has greatly improved the prognosis, most patients still need liver transplantation (LT) for long-term survival. The pathogenesis of BA has not been fully clarified, and liver fibrosis in BA is far beyond biliary obstructive cirrhosis.
Data sources: Literature reviews were underwent through PubMed. Persistent inflammation, immune response, biliary epithelial-mesenchymal transition, matrix deposition, decompensated angiogenesis, and unique biliary structure development all contribute to the fibrosis process. Observed evidences in such fields have been collected and form the backbone of this review.
Results: Interactions of the multiple pathways accelerate this process.
Conclusions: Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure.
Keywords: Biliary atresia; Children; Liver fibrosis.