Biliary Atresia-emerging diagnostic and therapy opportunities

Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of liver development with a focus on cholangiocyte differentiation and how use of technical platforms, including rodent, zebrafish and organoid models, advances our understanding of Biliary Atresia. This is followed by a description of potential pathomechanisms, such as autoimmune responses, inflammation, disturbed apical-basal cell polarity, primary cilia dysfunction as well as beta-amyloid accumulation. Finally, we describe current and emerging diagnostic opportunities and recent translation breakthroughs for Biliary Atresia in the area of emerging therapy development, including immunomodulation and organoid-based systems for liver and bile duct repair. (C) 2021 The Author(s). Published by Elsevier B.V.

Automated summary

This review discusses recent progress in understanding liver development and potential pathomechanisms of Biliary Atresia. It also highlights current and emerging diagnostic opportunities, as well as translation breakthroughs in therapy development for this devastating pediatric cholangiopathy.