title: 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: Department of Gastroenterology and Hepatology, Karolinska Institute, Karolinska University Hospital, Stockholm, Sweden. staffan.wahlin@karolinska.se
authors: Staffan
authors: S
authors: Wahlin
authors: Per
authors: P
authors: Stal
authors: Rene
authors: R
authors: Adam
authors: Vincent
authors: V
authors: Karam
authors: Robert
authors: R
authors: Porte
authors: Daniel
authors: D
authors: Seehofer
authors: Bridget K
authors: BK
authors: Gunson
authors: Jens
authors: J
authors: Hillingsø
authors: Jürgen L
authors: JL
authors: Klempnauer
authors: Jan
authors: J
authors: Schmidt
authors: Graeme
authors: G
authors: Alexander
authors: John
authors: J
authors: O'Grady
authors: Pierre-Alain
authors: PA
authors: Clavien
authors: Mauro
authors: M
authors: Salizzoni
authors: Andreas
authors: A
authors: Paul
authors: Keith
authors: K
authors: Rolles
authors: Bo-Göran
authors: BG
authors: Ericzon
authors: Pauline
authors: P
authors: Harper