title: [Severe liver failure in erythropoietic protoporphyria. A report of a case treated by liver transplantation].
abstract: Severe liver failure is a rare complication of erythropoietic protoporphyria (PEP), which is associated with a high rate mortality. Until now, 31 patients with this hepatic complication had underwent a liver transplantation, with a high rate of survival, but their long-term outcome is not well established. We report the first case in Spain of PEP in 59-year-old, whose acute liver failure was treated with liver transplantation, without postoperative complications. The patient is in good clinical condition 30 months later. Nevertheless during the first eleven months of follow-up the plasma levels of protoporphyrin remained elevated, which was accompanied of biochemical and histological evidence of relapse of the metabolic disease in the graft. Cases such as this stress the usefulness of liver transplantation, but also the need of more efficient measures to decrease the protoporphyrin levels before and after the transplant, in order to prevent hepatic and extrahepatic complications in these patients.
authors.affiliation: Servicio de Gastroenterología y Hepatología, Hospital Universitario Virgen Macarena, Sevilla.
authors.forename: M
authors.initials: M
authors.lastname: Jiménez-Sáenz
authors.forename: A
authors.initials: A
authors.lastname: Caunedo-Alvarez
authors.forename: M
authors.initials: M
authors.lastname: Rojas
authors.forename: M
authors.initials: M
authors.lastname: Mata
authors.forename: J L
authors.initials: JL
authors.lastname: Villar
authors.forename: A
authors.initials: A
authors.lastname: Piñar
authors.forename: J
authors.initials: J
authors.lastname: Herrerías-Gutiérrez
mesh_terms: D001706:Biopsy; D002779:Cholestasis / Q000209:etiology; D003131:Combined Modality Therapy; D003306:Coproporphyrins / Q000032:analysis; D006801:Humans; D008099:Liver / Q000473:pathology; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000473:pathology; D008297:Male; D008875:Middle Aged; D017121:Porphyria, Hepatoerythropoietic / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D011184:Postoperative Period; D011524:Protoporphyrins / Q000032:analysis; D012008:Recurrence; D000637:Transaminases / Q000032:analysis
chemical_list: D003306:Coproporphyrins; D011524:Protoporphyrins; D000637:Transaminases