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: Nathalie Stojeba, Carole Meyer, Catherine Jeanpierre, Frédéric Perrot, Christine Hirth, Thierry Pottecher, Jean-Charles Deybach
• Journal: Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society (Liver Transpl) Vol. 10 Issue 7 Pg. 935-8 (Jul 2004) ISSN: 1527-6465 [Print] United States
• PMID: 15237381 (Publication Type: Case Reports, Journal Article)

Topics

• Humans
• Liver Transplantation (methods)
• Male
• Middle Aged
• Porphyria, Variegate (surgery)
• Treatment Outcome