li-11-11            Idiopathic adulthood ductopenia            By Dr. E. Orfei


Vanishing bile duct syndrome with jaundice and pruritus which  lacks  all characteristics and antibodies

associated with other vanishing bile duct conditions.


Two types of this syndrome are recognized.

Type one include patients who are asymptomatic or present only cholestasis. They have less bile duct destruction

on liver biopsy and their clinical course ranges from spontaneous improvement to development of biliary cirrhosis.

Type two include those patients presenting decompensated biliary cirrhosis and have marked destruction of intrahepatic bile ducts  and require orthotopic liver transplantation which will  result in complete resolution of the jaundice and pruritus. (Arch Intern Med.160:1033, 2000).