[Liver transplantation for the treatment of type I familial amyloidotic polyneuropathy].
The first liver transplantation carried out in Spain for the treatment of type I familial amyloidotic polyneuropathy (FAP I) is presented. The reason for the operation was based on the liver being responsible for the synthesis of abnormal transtirretin (TTR) constituting the peculiar amyloid of the disease. Following transplantation a rapid and noticeable decrease in abnormal TTR was observed and the evolution of the clinical picture after 18 months of surgery is favorable with progressive improvement of the neurologic symptoms and normal function of the graft. These encouraging results coincide with those of the Swedish group of Umea, the pioneer of this procedure.
López Andreu
F R
FR
Servicio de Medicina Interna, Hospital General Universitario, Murcia.
Munar-Qués
M
M
Parrilla
P
P
Escribano Soriano
J B
JB
Costa
P P
PP
Costa
P M
PM
Almeida
M R
MR
Pons
J A
JA
Robles
R
R
Sánchez-Bueno
F
F
D000328:Adult; D017772:Amyloid Neuropathies / Q000097:blood / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D010375:Pedigree; D011228:Prealbumin / Q000378:metabolism
D011228:Prealbumin
Trasplante hepático para el tratamiento de la polineuropatía amiloidótica familiar tipo I.