Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years
| Autor: | Manuel Abradelo de Usera, Félix Cambra Molero, Carmelo Loinaz Segurola, Enrique Moreno González, Alejandro Manrique Municio, Iago Justo Alonso, Alberto Marcacuzco Quinto, Alvaro Garcia-Sesma Perez-F, Jorge Calvo Pulido, Oscar Caso M, Luis Carlos Jiménez Romero |
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| Rok vydání: | 2015 |
| Předmět: |
Adult
Male medicine.medical_specialty medicine.medical_treatment Liver transplantation Gastroenterology Internal medicine Humans Medicine In patient Aged Retrospective Studies Amyloid Neuropathies Familial biology business.industry Amyloidosis Autosomal dominant trait Middle Aged Familial amyloid neuropathy medicine.disease Liver Transplantation Surgery Organ damage Transthyretin biology.protein Amyloid polyneuropathy Female business |
| Zdroj: | Medicina Clínica (English Edition). 144:385-388 |
| ISSN: | 2387-0206 |
| Popis: | Background and objective Familial amyloid polyneuropathy (FAP) is the most prevalent type of hereditary systemic amyloidosis. It is an autosomal dominant disease characterized by the deposition of an abnormal variant transthyretin. It has a worldwide distribution, with localized endemic areas in Portugal, Sweden and Japan. In Spain there is an endemic focus, located in Mallorca. Liver transplantation is the only curative option for patients with FAP. The aim of this study was to describe the clinical and demographic characteristics of patients transplanted with a diagnosis of PAF. Material and method Six patients with PAF underwent liver transplantation between April 1986 and December 2012. Results The mean age was 57.7 + 16 years, patients of Spanish origin were older than 60 years. All patients had progressive symptoms as mixed polyneuropathy. In 2 patients, combined heart–liver transplants sequentially were performed. Patient survival and graft was 80% at 1, 3 and 5 years. Conclusions The only effective treatment for etiologic PAF is liver transplantation. Early detection is the key to the treatment and control, avoiding the irreversible organ damage. |
| Databáze: | OpenAIRE |
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