Recovery from a variegate porphyria by a liver transplantation.
Liver Transpl
; 10(7): 935-8, 2004 Jul.
Article
em En
| MEDLINE
| ID: mdl-15237381
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Transplante de Fígado
/
Porfiria Variegada
Limite:
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Liver Transpl
Assunto da revista:
GASTROENTEROLOGIA
/
TRANSPLANTE
Ano de publicação:
2004
Tipo de documento:
Article
País de afiliação:
França