Table 2.

Targets for Intervention

Increase protoporphyrin excretion into bile: ursodeoxycholic acid Reduce protoporphyrin production: hematin and red blood cell transfusion Decrease the circulating pool of protoporphyrin – Removal: plasmapheresis, hemodialysis, and exchange transfusions – Interrupting enterohepatic circulation: cholestyramine and activated charcoal Use antioxidants or cytoprotective agents: vitamin E and ursodeoxycholic acid Perform liver transplantation Correct the underlying defect: bone marrow transplantation