Liver transplantation for erythropoietic protoporphyria in Europe.

Abstract

Liver transplantation is an established lifesaving treatment for patients with severe protoporphyric liver disease, but disease recurrence in the graft occurs for the majority of recipients. Severe burn injuries may occur when protective light filters are not used with surgical luminaires. Motor neuropathy with an unclear pathogenesis is a frequent complication. We retrospectively studied 35 transplants performed for protoporphyric liver disease in 31 European patients between 1983 and 2008. Most of the patients were male (61.3%), and the mean age at the time of primary transplantation was 39 years (range = 9-60 years). The overall patient survival rates were 77% at 1 year and 66% at 5 and 10 years. The overall rate of disease recurrence in the graft was 69%. Forty-three percent of the patients experienced recurrence within a year, but this was often a transient finding that was associated with other graft complications. Phototoxic injuries due to surgical luminaires were seen in 25.0% of the patients who were not protected by filters, but these injuries were not seen in the 9 patients who were protected by filters. Significant motor neuropathies requiring prolonged ventilation complicated the postoperative course for 5 of the 31 patients (16.1%). Hematopoietic stem cell transplantation was performed for 3 patients to prevent graft loss due to disease recurrence. Prognostic markers are needed to identify patients prone to severe protoporphyric liver disease so that curative stem cell transplantation can be offered to select patients instead of liver transplantation.

• Authors: Staffan Wahlin, Per Stal, Rene Adam, Vincent Karam, Robert Porte, Daniel Seehofer, Bridget K Gunson, Jens Hillingsø, Jürgen L Klempnauer, Jan Schmidt, Graeme Alexander, John O'Grady, Pierre-Alain Clavien, Mauro Salizzoni, Andreas Paul, Keith Rolles, Bo-Göran Ericzon, Pauline Harper, European Liver and Intestine Transplant Association
• Journal: Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society (Liver Transpl) Vol. 17 Issue 9 Pg. 1021-6 (Sep 2011) ISSN: 1527-6473 [Electronic] United States
• PMID: 21604355 (Publication Type: Journal Article)
• Copyright: Copyright © 2011 American Association for the Study of Liver Diseases.

Topics

• Adolescent
• Adult
• Child
• Demography
• Europe
• Female
• Humans
• Liver Failure (mortality, therapy)
• Liver Transplantation (methods)
• Male
• Middle Aged
• Protoporphyria, Erythropoietic (mortality, therapy)
• Recurrence
• Retrospective Studies
• Risk
• Stem Cell Transplantation (methods)
• Treatment Outcome