Liver histology. A: liver histology in a PFIC1 patient at age six months. It shows moderate lobular and portal fibrosis; B: liver histology in a PFIC2 patient at age 11 months. It shows hepatocellular necrosis, giant cell transformation and cirrhosis; C: liver histology in a PFIC3 patient at age 10 months. It shows strong (true) ductular proliferation within the all portal tract; D: at age 5 years in the same PFIC3 patient, there is a typical picture of biliary cirrhosis with ductules containing bile plugs, while patent biliary tree.