Biliary disease in children-up to date-

Abstract

Biliary disease in children is caused by abnormalities in bile composition, biliary anatomy, or function. Novel causes in genetic cholestatic disease and neonatal sclerosing cholangitis have been detected by genetic analysis. Research is advancing on primary sclerosing cholangitis regarding with dysbiosis of the gut microbiota, as well as ducal plate malformation. The natural history and long-term prognosis, which are currently poorly understood, are being revealed through world-wide multicenter and disease-specific registry. Pharmaceutical research has been done on pediatric children with biliary diseases; ileal bile acid transporter inhibitors have been shown to improve clinical outcomes in Alagille syndrome. Further pathophysiological understanding of biliary disease in children is expected to lead to therapeutic development.