Semin Liver Dis 1998;18(3):263-9

The intrahepatic cholangiopathies

Birnbaum A, Suchy FJ
Department of Pediatrics, Mount Sinai School of Medicine, New York, New York
10029, USA.

Abstract
The intrahepatic biliary epithelial cells or cholangiocytes are the primary
focus of injury in many congenital and acquired liver diseases of childhood.
Although cholangiocytes account for only 3 to 5% of the liver cell population,
injury and progressive loss of intrahepatic bile ducts can result in
considerable morbidity and mortality. Table 1 provides an overview of the
various disorders that affect the intrahepatic biliary tree. The more common
disorders are discussed in detail below. One of the most important
cholangiopathies, biliary atresia, characterized by complete destruction of the
extrahepatic biliary tree, with variable involvement of the intrahepatic bile
ducts, is discussed elsewhere in this series of articles. There has been
considerable progress in our understanding of the embryology and physiology of
the intrahepatic biliary system. These topics are also selectively reviewed,
with an emphasis on advances that aid in the understanding of the
pathophysiology of the disorders which affect the biliary tract in children