Fig. 3

Paucity of intrahepatic bile ducts in a patient with nephronophthisis (case 14). a-b The liver shows thin delicate portal/periportal fibrosis and eccentrically located portal veins with dilatation and herniation. Bile duct is missing in approximately 68% portal tracts. c-d In one portal tract there are residual bile ducts highlighted by CK19 immunohistochemistry, somewhat resembling ductal plate malformation. Magnification: a-b: 100X, c-d: 200x