title: [Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years].
abstract: 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.
  
  
  METHODS
  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 one, 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.
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España. Electronic address: alejandro_mq@yahoo.es.
authors.forename: Alberto A
authors.initials: AA
authors.lastname: Marcacuzco Quinto
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Alejandro
authors.initials: A
authors.lastname: Manrique Municio
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Luis C
authors.initials: LC
authors.lastname: Jimenez Romero
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Carmelo
authors.initials: C
authors.lastname: Loinaz Segurola
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Jorge
authors.initials: J
authors.lastname: Calvo Pulido
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Iago
authors.initials: I
authors.lastname: Justo Alonso
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Alvaro
authors.initials: A
authors.lastname: Garcia-Sesma Perez-F
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Manuel
authors.initials: M
authors.lastname: Abradelo de Usera
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Felix
authors.initials: F
authors.lastname: Cambra Molero
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Oscar
authors.initials: O
authors.lastname: Caso M
authors.affiliation: Servicio de Cirugía General C y Trasplante de Órganos Abdominales, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Enrique
authors.initials: E
authors.lastname: Moreno Gonzalez
mesh_terms: D000328:Adult; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000601:surgery*; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
keywords: Amiloidosis; Amyloidosis; Familial amyloid neuropathy; Liver transplantation; Polineuropatía amiloidótica familiar; Trasplante hepático