Jornal de Hepatologia e Distúrbios Gastrointestinais
Acesso livre

Abstrato

Transplante hepático para tratamento da porfiria aguda intermitente: relato de um caso

Matthew R Kappus, Bryant B Summers, Jennifer S Byrns, Carl L Berg e 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.