作者
Henkjan J Verkade, Jorge A Bezerra, Mark Davenport, Richard A Schreiber, Georgina Mieli-Vergani, Jan B Hulscher, Ronald J Sokol, Deirdre A Kelly, Benno Ure, Peter F Whitington, Marianne Samyn, Claus Petersen
发表日期
2016/9/1
来源
Journal of hepatology
卷号
65
期号
3
页码范围
631-642
出版商
Elsevier
简介
Biliary Atresia and other cholestatic childhood diseases are rare conditions affecting the function and/or anatomy along the canalicular-bile duct continuum, characterised by onset of persistent cholestatic jaundice during the neonatal period. Biliary atresia (BA) is the most common among these, but still has an incidence of only 1 in 10–19,000 in Europe and North America. Other diseases such as the genetic conditions, Alagille syndrome (ALGS) and Progressive Familial Intrahepatic Cholestasis (PFIC), are less common. Choledochal malformations are amenable to surgical correction and require a high index of suspicion. The low incidence of such diseases hinder patient-based studies that include large cohorts, while the limited numbers of animal models of disease that recapitulate the spectrum of disease phenotypes hinders both basic research and the development of new treatments. Despite their individual …
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