The condition is usually<unk> , 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.
Congenital hepatic fibrosis
Neonatal cholestasis
Vanishing bile duct syndrome
Intrahepatic cholestasis of pregnancy
Progressive familial intrahepatic cholestasis
Autoimmune hepatitis
Polycystic liver disease
Biliary atresia