<unk>   is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and<unk>  that do not alter hepatic lobular architecture.  The<unk>  would affect resistance in portal veins leading to portal hypertension. The condition usually has an autosomal recessive pattern of transmission but sporadic cases have also been reported. It may be associated with other<unk>  defects; commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation.
Embryogenically<unk>  is due to malformation of duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive hepatocytes, which differentiate into cholangiocytes. It usually present in adolescent or young adulthood, but onset of sign and symptom can range from early childhood to 5th or 6th decade of life. Clinical features may vary but commonly include Cholangitis, hepatomegaly and signs of portal hypertension.
Congenital hepatic fibrosis
Benign intrahepatic cholestasis
Intrahepatic cholestasis of pregnancy
Progressive familial intrahepatic cholestasis
Autoimmune hepatitis
Biliary atresia
Hepatic veno-occlusive disease with immunodeficiency
Primary biliary cirrhosis