Signals for Hepatic Figrogenesis in Pediatric Cholestatic Liver Disease: Review and Hypothesis

Neonatal Cholestatic Liver Diseases Cholestatic liver disease in children occurs as a result of either an alteration in hepatocyte bile formation or disruption of bile flow out of the hepatocyte through intrahepatic bile ductules or extrahepatic bile ducts [1]. Liver disease usually appears within the first few weeks following birth. A large number of disorders exhibit cholestatic jaundice in neonatal life including (a) numerous cholangiopathies, such as extrahepatic biliary atresia, cystic fibrosis (CF), choledochal cyst, alpha1-Antitrypsin deficiency and Alagille's syndrome, (b) several abnormalities of the gall bladder, such as cholelithiasis and cholecystitis, and (c) bile acid transport disorders. The most commonly occurring form of neonatal cholestasis is biliary atresia, representing a relative frequency of approximately 30% [1]. In order to administer effective therapeutic intervention early diagnosis is critical. This can prove difficult as a number of phenotypic manifestations of the many different forms of neonatal cholestasis are similar and may even overlap.