Congenital hepatic fibrosis is predominantly associated with which cystic disease of kidney?: (A) Autosomal dominant polycystic kidney disease (ADPKD), (B) Autosomal recessive polycystic kidney disease (ARPKD), (C) Medullary sponge kidney, (D) Nephronophthisis
Answer is B. In Autosomal recessive polycystic kidney disease (ARPKD), patients who survive infancy may develop a peculiar hepatic injury characterized by:- Bland peripoal fibrosis Proliferation of well differentiated biliary ductules, now termed as congenital hepatic fibrosis.