Liver Transpl. 2004 Jul;10(7):935-8.

Recovery from a variegate porphyria by a liver transplantation.

Stojeba N, Meyer C, Jeanpierre C, Perrot F, Hirth C, Pottecher T, Deybach JC.

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Department of Anesthesiology, Hôpital de Hautepierre, Strasbourg, France. Nathalie.Stojeba@chru-strasbourg.fr

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.

PMID:: 15237381; [PubMed - indexed for MEDLINE]

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