title: Liver transplantation for erythropoietic protoporphyria with hepatic failure: a case report.
abstract: Erythropoietic protoporphyria (EPP) is a disorder of heme synthesis in which deficient ferrochelatase activity leads to excessive production and biliary excretion of protoporphyrin. The main clinical features--photosensitivity and hepatobiliary disease that may progress to liver failure--are caused by the toxicity of protoporphyrin. Orthotopic liver transplantation is an effective treatment of liver failure caused by EPP. In this report we have described an EPP Chinese man with end-stage liver disease. He was successfully transplanted. A 3-year follow-up study of protoporphyrin levels, liver tests, and liver biopsies showed no EPP recurrence after liver transplantation.
authors.affiliation: Key Laboratory of Living Donor Liver Transplantation, Ministry of Public Health, Liver Transplantation Center, First Affiliated Hospital of Nanjing Medical University, China.
authors.forename: F
authors.initials: F
authors.lastname: Zhang
authors.forename: L
authors.initials: L
authors.lastname: Lu
authors.forename: X
authors.initials: X
authors.lastname: Qian
authors.forename: L Y
authors.initials: LY
authors.lastname: Pu
authors.forename: G Q
authors.initials: GQ
authors.lastname: Li
authors.forename: X H
authors.initials: XH
authors.lastname: Wang
mesh_terms: D000328:Adult; D004890:Erythema / Q000209:etiology; D005500:Follow-Up Studies; D006801:Humans; D008027:Light / Q000009:adverse effects; D017093:Liver Failure / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D046351:Protoporphyria, Erythropoietic / Q000601:surgery*; D016896:Treatment Outcome