Liver transplantation for erythropoietic protoporphyria in Europe
Open Access
- 20 May 2011
- journal article
- research article
- Published by Ovid Technologies (Wolters Kluwer Health) in Liver Transplantation
- Vol. 17 (9), 1021-1026
- https://doi.org/10.1002/lt.22341
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. Liver Transpl 17:1021–1026, 2011. © 2011 AASLD.Keywords
This publication has 21 references indexed in Scilit:
- Delayed immune recovery following sequential orthotopic liver transplantation and haploidentical stem cell transplantation in erythropoietic protoporphyriaPediatric Transplantation, 2010
- Biliary complications following liver transplantation in the model for end-stage liver disease era: Effect of donor, recipient, and technical factorsLiver Transplantation, 2007
- Liver transplantation for erythropoietic protoporphyria liver diseaseLiver Transplantation, 2005
- Porphyrins, porphyrin metabolism and porphyrias. IV. Pathophysiology of erythyropoietic protoporphyria - diagnosis, care and monitoring of the patientScandinavian Journal of Clinical and Laboratory Investigation, 2000
- Long-term follow-up after liver transplantation for erythropoietic protoporphyriaEuropean Journal of Gastroenterology & Hepatology, 1999
- Follow-up after liver transplantation for protoporphyric liver diseaseLiver Transplantation and Surgery, 1996
- Evidence for neurological dysfunction in end-stage protoporphyric liver diseaseHepatology, 1993
- Erythropoietic protoporphyria: Unusual skin and neurological problems after liver transplantationGastroenterology, 1991
- Predictable and unpredictable hazards of erythropoietic protoporphyriaClinical and Experimental Dermatology, 1991
- Hepatobiliary implications and complications in protoporphyria, a 20-year studyClinical Biochemistry, 1989