| Recovery from a variegate porphyria by a liver transplantation. | |
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MedLine Citation:
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PMID: 15237381 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Nathalie Stojeba; Carole Meyer; Catherine Jeanpierre; Frédéric Perrot; Christine Hirth; Thierry Pottecher; Jean-Charles Deybach |
Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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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:
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Created Date: 2004-07-06 Completed Date: 2005-03-07 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 100909185 Medline TA: Liver Transpl Country: United States |
Other Details:
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Languages: eng Pagination: 935-8 Citation Subset: IM |
Affiliation:
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Department of Anesthesiology, Hôpital de Hautepierre, Strasbourg, France. Nathalie.Stojeba@chru-strasbourg.fr |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Humans Liver Transplantation* / methods Male Middle Aged Porphyria, Variegate / surgery* Treatment Outcome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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