title: Recovery from a variegate porphyria by a liver transplantation.
abstract: The porphyrias are a group of inherited or acquired enzymatic defects of heme biosynthesis. Each type of porphyria has a characteristic pattern of overproduction and accumulation of heme precursors based on the location of dysfunctional enzyme in the heme synthetic pathway. Variegate porphyria, one of the acute hepatic porphyrias, is characterized by a partial reduction in protoporphyrinogen oxidase, the seventh enzyme of the heme biosynthetic pathway. A case of liver transplantation is described with a recovery from a variegate porphyria. Acute porphyria is commonly worsened by a wide variety of medications. We describe a step-by-step perioperative management protocol.
authors.affiliation: Department of Anesthesiology, Hôpital de Hautepierre, Strasbourg, France. Nathalie.Stojeba@chru-strasbourg.fr
authors.forename: Nathalie
authors.initials: N
authors.lastname: Stojeba
authors.forename: Carole
authors.initials: C
authors.lastname: Meyer
authors.forename: Catherine
authors.initials: C
authors.lastname: Jeanpierre
authors.forename: Frédéric
authors.initials: F
authors.lastname: Perrot
authors.forename: Christine
authors.initials: C
authors.lastname: Hirth
authors.forename: Thierry
authors.initials: T
authors.lastname: Pottecher
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Deybach
mesh_terms: D006801:Humans; D016031:Liver Transplantation / Q000379:methods; D008297:Male; D008875:Middle Aged; D046350:Porphyria, Variegate / Q000601:surgery*; D016896:Treatment Outcome