Liver Transplantation because of Acute Liver Failure due to Heme Arginate Overdose in a Patient with Acute Intermittent Porphyria

• Main Authors: Pascal Frei, Elisabeth I. Minder, Natascia Corti, Beat Muellhaupt, Andreas Geier, Heiner Adams, Jean-Paul Dutertre, Alain Rudiger, Philipp Dutkowski, Marco Maggiorini, Christoph C. Ganter
• Format: Article
• Language: English
• Published: Karger Publishers 2012-04-01
• Series: Case Reports in Gastroenterology
• Subjects: Heme arginate; Acute intermittent porphyria; Overdose; Acute liver failure; Liver transplantation
• Online Access: http://www.karger.com/Article/FullText/338354

In acute attacks of acute intermittent porphyria, the mainstay of treatment is glucose and heme arginate administration. We present the case of a 58-year-old patient with acute liver failure requiring urgent liver transplantation after erroneous 6-fold overdose of heme arginate during an acute attack. As recommended in the product information, albumin and charcoal were administered and hemodiafiltration was started, which could not prevent acute liver failure, requiring super-urgent liver transplantation after 6 days. The explanted liver showed no preexisting liver cirrhosis, but signs of subacute liver injury and starting regeneration. The patient recovered within a short time. A literature review revealed four poorly documented cases of potential hepatic and/or renal toxicity of hematin or heme arginate. This is the first published case report of acute liver failure requiring super-urgent liver transplantation after accidental heme arginate overdose. The literature and recommendations in case of heme arginate overdose are summarized. Knowledge of a potentially fatal course is important for the management of future cases. If acute liver failure in case of heme arginate overdose is progressive, super-urgent liver transplantation has to be evaluated.