Embryogenically,<unk>  is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive<unk> ytes, which differentiate into cholangiocytes.<unk>  usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include cholangitis, hepatomegaly and signs of portal hypertension.
Congenital hepatic fibrosis
Neonatal cholestasis
Vanishing bile duct syndrome
Intrahepatic cholestasis of pregnancy
Progressive familial intrahepatic cholestasis
Autoimmune hepatitis
Polycystic liver disease
Biliary atresia