Liver transplantation and the management of progressive familial intrahepatic cholestasis in children

Figure 5 Clinical presentation of progressive familial intrahepatic cholestasis type 3. A: Progressive familial intrahepatic cholestasis type 3 (PFIC3) has a variable clinical presentation and may show nonspecific biliary pattern of injury that can mimic extrahepatic biliary atresia such as bile duct proliferation and cholestasis. In this patient with PFIC3 there is cholestasis, inflammation, and bile duct proliferation; B: Biliary type cirrhosis in a patient with PFIC3 with severe cholestasis (arrow) and micronodular cirrhosis.