Liver Transplantation for Familial Amyloidotic Polyneuropathy (FAP): A Single‐Center Experience Over 16 Years
| Autor: | Shinji Yamamoto, Henryk Wilczek, Ole B. Suhr, Yukio Ando, Greg Nowak, Antti Oksanen, B-G Ericzon, Henrik Gjertsen, Lars Wikström, Takashi Iwata, Gunnar Söderdahl, M. Larsson |
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| Rok vydání: | 2007 |
| Předmět: |
Adult
Male congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty medicine.medical_treatment Nutritional Status Liver transplantation Single Center Gastroenterology Internal medicine Humans Immunology and Allergy Medicine Pharmacology (medical) Age of Onset Metabolic disease neoplasms Aged Retrospective Studies Amyloid Neuropathies Familial Transplantation biology business.industry Amyloidosis Graft Survival Follow up studies Middle Aged medicine.disease Survival Analysis digestive system diseases Liver Transplantation Surgery Transthyretin surgical procedures operative biology.protein Female Age of onset business Polyneuropathy Follow-Up Studies |
| Zdroj: | American Journal of Transplantation. 7:2597-2604 |
| ISSN: | 1600-6135 |
| DOI: | 10.1111/j.1600-6143.2007.01969.x |
| Popis: | Orthotopic liver transplantation (LTx) is currently the only available treatment that has been proven to halt the progress of familial amyloidotic polyneuropathy (FAP). The aim of this study was to assess mortality and symptomatic response to LTx for FAP. All 86 FAP patients transplanted at our hospital between April 1990 and November 2005 were included in the study. Five patients underwent retransplantation. The 1-, 3- and 5-year patient survival rates in patients transplanted during 1996-2005 were 94.6%, 92.3% and 92.3%, respectively, a significant difference from the rates of 76.7%, 66.7% and 66.7%, respectively, during 1990-1995 (p = 0.0003). Multivariate analysis revealed that the age at the time of LTx (or=40 years), duration of the disease (or=7 years) and modified body mass index (mBMI) (600) were independent prognostic factors for patient survival. A halt in the progress of symptoms was noted in most patients, but only a minority experienced an improvement after LTx. To optimize the posttransplant prognosis, LTx should be performed in the early stages of the disease, and close post-LTx monitoring of heart function by echocardiography and of heart arrhythmia by Holter ECG is mandatory. |
| Databáze: | OpenAIRE |
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