Nonsyndromic paucity of intrahepatic bile ducts in infancy and idiopathic ductopenia in adulthood: The same syndrome?
Miquel Bruguera, Josep Llach, Joan Rodés – 1 May 1992 – Eleven patients with chronic intrahepatic cholestasis in whom a liver biopsy specimen showed unexplained bile duct paucity were investigated. Cholestasis developed during the neonatal period in three, at infancy (before 14 yr) in four and after 14 yr in the remaining four patients. In all patients other conditions characterized by chronic cholestasis associated with ductopenia such as primary biliary cirrhosis, primary sclerosing cholangitis, drug‐induced liver disease, sarcoidosis or graft‐vs.‐host disease were excluded.