DCDC2 and polycystic kidney disease 4: More specifically, the renal-hepatic ciliopathy due to Doublecortin domain-containing protein 2 (DCDC2) mutations results in a hepatic histological pattern resembling congenital hepatic fibrosis, but high GGT cholestasis in this condition appears independent from biliary ducts infections but rather is linked to sclerosing cholangitis.