Primary cilia, antenna-like sensory organelles protruding from the surface of most vertebrate
cell types, are essential for regulating signalling pathways during development and adult …

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 …

Cholestasis is the predominate feature of many pediatric hepatobiliary diseases. The
physiologic flow of bile requires multiple complex processes working in concert. Bile acid …

With the application of modern investigative technologies, cholestatic liver diseases of
genetic etiology are increasingly identified as the root cause of previously designated …

Biliary atresia (BA) is an obliterative condition of the biliary tract that presents with persistent
jaundice and pale stools typically in the first few weeks of life. While this phenotypic …

Biliary atresia is a rare disease but remains the most common indication for pediatric liver
transplantation as there are no effective medical therapies to slow progression after …

Background & Aims Biliary atresia (BA) is poorly understood and leads to liver
transplantation (LT), with the requirement for and associated risks of lifelong …

Conclusions: Bile duct ligation of the Pkd1l1-deficient mouse model mirrors several aspects
of the intrahepatic pathophysiology of biliary atresia in humans including bile duct …

The primary cilium is increasingly recognized as a crucial player in the physiology of biliary
epithelial cells (BECs). However, the precise role of primary cilia in the development of age …

Chronic cholestasis due to heritable causes is usually diagnosed in childhood. However,
many cases can present and survive into adulthood. The time course varies considerably …