Journal d'hépatologie et des troubles gastro-intestinaux
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Transplantation hépatique pour la prise en charge de la porphyrie aiguë intermittente : rapport de cas

Matthew R. Kappus, Bryant B. Summers, Jennifer S. Byrns, Carl L. Berg et Julius M. Wilder

We report a single patient with acute intermittent porphyria whose porphyria was successfully treated with orthotopic liver transplantation. She had a very poor quality of life as a result of years of frequent acute porphyria symptoms manifesting as abdominal pain crises. After transplantation, clinical signs of porphyria resolved as expected. This case adds to a growing body of literature which is assisting to formulate the indications for, and the timing of, transplantation for AIP.