Recovery from a variegate porphyria by a liver transplantation.
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.
Stojeba
Nathalie
N
Department of Anesthesiology, Hôpital de Hautepierre, Strasbourg, France. Nathalie.Stojeba@chru-strasbourg.fr
Meyer
Carole
C
Jeanpierre
Catherine
C
Perrot
Frédéric
F
Hirth
Christine
C
Pottecher
Thierry
T
Deybach
Jean-Charles
JC
D006801:Humans; D016031:Liver Transplantation / Q000379:methods; D008297:Male; D008875:Middle Aged; D046350:Porphyria, Variegate / Q000601:surgery*; D016896:Treatment Outcome