Biliary Atresia – emerging diagnostic and therapy opportunities
Urban Lendahl,
Vincent C.H. Lui,
Patrick H.Y. Chung,
Paul K.H. Tam
Affiliations
Urban Lendahl
Department of Cell and Molecular Biology, Karolinska Institutet, Stockholm, Sweden; Dr. Li Dak-Sum Research Centre, the University of Hong Kong, Hong Kong; CORRESPONDING AUTHORS: Paul KH Tam, Division of Paediatric Surgery, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong, Tel: (852) 2255 4850; Fax: (852) 2817 3155
Vincent C.H. Lui
Dr. Li Dak-Sum Research Centre, the University of Hong Kong, Hong Kong; Department of Surgery, Li Ka Shing Faculty of Medicine, the University of Hong Kong, Hong Kong
Patrick H.Y. Chung
Department of Surgery, Li Ka Shing Faculty of Medicine, the University of Hong Kong, Hong Kong; Department of Surgery, University of Hong Kong-Shenzhen Hospital, Shenzhen, Guangdong, China
Paul K.H. Tam
Dr. Li Dak-Sum Research Centre, the University of Hong Kong, Hong Kong; Department of Surgery, Li Ka Shing Faculty of Medicine, the University of Hong Kong, Hong Kong; Department of Surgery, University of Hong Kong-Shenzhen Hospital, Shenzhen, Guangdong, China; CORRESPONDING AUTHORS: Paul KH Tam, Division of Paediatric Surgery, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong, Tel: (852) 2255 4850; Fax: (852) 2817 3155
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.
