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Biliary Atresia - emerging diagnostic and therapy opportunities.
Lendahl, Urban; Lui, Vincent C H; Chung, Patrick H Y; Tam, Paul K H.
Afiliación
  • Lendahl U; Department of Cell and Molecular Biology, Karolinska Institutet, Stockholm, Sweden; Dr. Li Dak-Sum Research Centre, the University of Hong Kong, Hong Kong. Electronic address: urban.lendahl@ki.se.
  • Lui VCH; 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.
  • Chung PHY; 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.
  • Tam PKH; 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. Electronic address: paultam@hku.hk.
EBioMedicine ; 74: 103689, 2021 Dec.
Article en En | MEDLINE | ID: mdl-34781099
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Conductos Biliares / Atresia Biliar / Organoides Tipo de estudio: Diagnostic_studies / Prognostic_studies Límite: Animals / Humans Idioma: En Revista: EBioMedicine Año: 2021 Tipo del documento: Article Pais de publicación: Países Bajos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Conductos Biliares / Atresia Biliar / Organoides Tipo de estudio: Diagnostic_studies / Prognostic_studies Límite: Animals / Humans Idioma: En Revista: EBioMedicine Año: 2021 Tipo del documento: Article Pais de publicación: Países Bajos