Document Detail

Recovery from a variegate porphyria by a liver transplantation.
MedLine Citation:
PMID:  15237381     Owner:  NLM     Status:  MEDLINE    
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.
Nathalie Stojeba; Carole Meyer; Catherine Jeanpierre; Frédéric Perrot; Christine Hirth; Thierry Pottecher; Jean-Charles Deybach
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society     Volume:  10     ISSN:  1527-6465     ISO Abbreviation:  Liver Transpl.     Publication Date:  2004 Jul 
Date Detail:
Created Date:  2004-07-06     Completed Date:  2005-03-07     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100909185     Medline TA:  Liver Transpl     Country:  United States    
Other Details:
Languages:  eng     Pagination:  935-8     Citation Subset:  IM    
Department of Anesthesiology, Hôpital de Hautepierre, Strasbourg, France.
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MeSH Terms
Liver Transplantation* / methods
Middle Aged
Porphyria, Variegate / surgery*
Treatment Outcome

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