Copyright copy;2010 Baishideng Publishing Group Co.
World J Gastroenterol. Sep 28, 2010; 16(36): 4526-4531
Published online Sep 28, 2010. doi: 10.3748/wjg.v16.i36.4526
Table 1 Pathogenic mechanisms and therapeutic approaches of erythropoietic protoporphyria
Pathogenic mechanismTreatment
Induce bile flowUrsodeoxycholic acid
Reduce protoporphyrin productionParenteral iron
Transfusion of erythrocytes
Infusions of hematin
Reduce protoporphyrin levelsPlasmapheresis
Extracorporeal albumin dialysis
Interrupt enterohepatic circulationCholestyramine
Activated charcoal
Protect hepatocytes from toxic damageN-acetyl cysteine
Remove the principal source of protoporphyrinBone marrow transplant
Erythropoietic protoporphyria-related liver failureLiver transplant