RT Journal Article
T1 Trasplante hepático como tratamiento de la polineuropatía amiloidótica familiar en pacientes mayores de 60 años
T2 Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years
A1 Marcacuzco Quinto, Alberto Alejandro
A1 Manrique Municio, Alejandro
A1 Jiménez Romero, Luis Carlos
A1 Loinaz Segurola, Carmelo
A1 Calvo Pulido, Jorge
A1 Justo Alonso, Iago
A1 García-Sesma Pérez-Fuentes, Álvaro
A1 Abradelo De Usera, Manuel
A1 Cambra Molero, Félix
A1 Caso Maestro, Óscar
A1 Moreno González, Enrique
AB 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 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.
PB Elsevier
SN 0025-7753
YR 2015
FD 2015-05-08
LK https://hdl.handle.net/20.500.14352/98843
UL https://hdl.handle.net/20.500.14352/98843
LA spa
DS Docta Complutense
RD 6 abr 2025