Biliary Atresia - emerging diagnostic and therapy opportunities

EBioMedicine. 2021 Dec:74:103689. doi: 10.1016/j.ebiom.2021.103689. Epub 2021 Nov 12.


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.

Keywords: Biliary Atresia; Cholangiocyte; Cholangiopathy; Disease Models; Pathomechanisms; Therapy.

Publication types

  • Review

MeSH terms

  • Animals
  • Bile Ducts / cytology*
  • Biliary Atresia / diagnosis*
  • Biliary Atresia / pathology
  • Cell Differentiation
  • Disease Models, Animal
  • Epithelial Cells / cytology
  • Humans
  • Organoids / pathology*