Table 4.
PFIC type 1 | PFIC type 2 | PFIC type 3 | |
---|---|---|---|
Histology | Intracanalicular cholestasis, bile duct proliferation at the late phase | Bile duct proliferation at the late phase, lobular and portal fibrosis | Extensive bile duct proliferation and periportal fibrosis |
Bile on electronmicroscopy | Granular called “Byler bile” | Amorphous or filamentous | _ |
Therapy | UDCA biliary diversion liver transplantation | UDCA biliary diversion liver transplantation | UDCA liver transplantation |
Abbreviations: PFIC, progressive familial intrahepatic cholestasis; UDCA, ursodeoxycholic acid.