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Long-term consequences of domino liver transplantation using familial amyloidotic polyneuropathy grafts
Authors:Shinji Yamamoto  Henryk E Wilczek  Takashi Iwata  Marie Larsson  Henrik Gjertsen  Gunnar Söderdahl  Göran Solders  Bo-Göran Ericzon
Institution:Division of Transplantation surgery, Department of Clinical Science, Intervention and Technology, Karolinska University Hospital, Huddinge, Stockholm, Sweden. shinji.yamamoto@ki.se
Abstract:Domino liver transplantation (DLT) using grafts from patients with familial amyloidotic polyneuropathy (FAP) is an established procedure at many transplantation centers. However, data evaluating the long-term outcome of DLT are limited. The aim of the present study was to analyze the risk of de novo polyneuropathy, possibly because of amyloidosis, and the patient survival after DLT. At our department, 28 DLT using FAP grafts were conducted between January 1997 and December 2005. One patient was twice subjected to DLT. Postoperative neurological monitoring of peripheral nerve function was performed with electroneurography (ENeG) in 20 cases. An ENeG index based on 12 parameters was calculated and correlated to age and/or height. Three patients developed ENeG signs of polyneuropathy 2-5 years after the DLT, but with no clinical symptoms. The 1-, 3- and 5-year actuarial patient survival in hepatocellular carcinoma (HCC) patients (n = 12) and non-HCC patients (n = 15) was 67%, 15%, 15% and 93%, 93%, 80%, respectively (P = 0.001). Development of impaired nerve conduction in a proportion of patients may indicate that de novo amyloidosis occurs earlier than previously expected. Survival after DLT was excellent except in patients with advanced HCC.
Keywords:domino transplantation  familial amyloidotic polyneuropathy  outcome
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