Erythropoietic protoporphyria and pretransplantation treatment with nonbiological liver assist devices
Martin Eefsen, Allan Rasmussen, Hans Christian Wulf, Axel Brock, Bent Adel Hansen – 20 March 2007 – Erythropoietic protoporphyria (EPP) is a disease of the heme metabolism due to a deficiency of ferrochelatase, leading to accumulation of protoporphyrin (PPIX) in the erythrocyte (red blood cell [RBC]). The major clinical manifestation in EPP is photosensitivity; however, in a small number of patients liver failure is a significant complication and liver transplantation is the only treatment option.