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Titolo:
The course and prognostic factors of familial amyloid polyneuropathy afterliver transplantation
Autore:
Adams, D; Samuel, D; Goulon-Goeau, C; Nakazato, M; Costa, PMP; Feray, C; Plante, V; Ducot, B; Ichai, P; Lacroix, C; Metral, S; Bismuth, H; Said, G;
Indirizzi:
Hop Bicetre, Serv Neurol, F-94275 Le Kremlin Bicetre, France Hop Bicetre Le Kremlin Bicetre France F-94275 Le Kremlin Bicetre, France Hop Bicetre, Lab Louis Ranvier, F-94275 Le Kremlin Bicetre, France Hop Bicetre Le Kremlin Bicetre France F-94275 Le Kremlin Bicetre, France Hop Bicetre, Serv Explorat Fonct Syst Nerveux, F-94275 Le Kremlin Bicetre,France Hop Bicetre Le Kremlin Bicetre France F-94275 Le Kremlin Bicetre,France Hop Bicetre, Dept Epidemiol, INSERM, U292, F-94275 Le Kremlin Bicetre, France Hop Bicetre Le Kremlin Bicetre France F-94275 Le Kremlin Bicetre, France Hop Paul Brousse, Ctr Hepatobiliaire, Villejuif, France Hop Paul Brousse Villejuif France Ctr Hepatobiliaire, Villejuif, France Univ Paris Sud, Assistance Publ Hop Paris, Paris, France Univ Paris Sud Paris France d, Assistance Publ Hop Paris, Paris, France Miyazaki Med Coll, Dept Internal Med 3, Miyazaki 88916, Japan Miyazaki MedColl Miyazaki Japan 88916 rnal Med 3, Miyazaki 88916, Japan Ctr Estudos Paramyloidosis, Porto, Portugal Ctr Estudos Paramyloidosis Porto Portugal ramyloidosis, Porto, Portugal
Titolo Testata:
BRAIN
, volume: 123, anno: 2000,
parte:, 7
pagine: 1495 - 1504
SICI:
0006-8950(200007)123:<1495:TCAPFO>2.0.ZU;2-G
Fonte:
ISI
Lingua:
ENG
Soggetto:
TRANSTHYRETIN MESSENGER-RNA; IMPROVEMENT; PROGRESSION; NEUROPATHY; RAT;
Keywords:
familial amyloid polyneuropathy; liver transplantation; transthyretin mutation; course;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
27
Recensione:
Indirizzi per estratti:
Indirizzo: Adams, D Hop Bicetre, Serv Neurol, F-94275 Le Kremlin Bicetre, France Hop Bicetre Le Kremlin Bicetre France F-94275 n Bicetre, France
Citazione:
D. Adams et al., "The course and prognostic factors of familial amyloid polyneuropathy afterliver transplantation", BRAIN, 123, 2000, pp. 1495-1504

Abstract

Familial amyloid polyneuropathy (FAP) associated with mutations of the transthyretin (TTR) gene is the most common type of FAP, a devastating diseasecausing death within 10 years after the first symptoms. Because most of the amyloidogenic mutated TTR is secreted by the li,er, transplantation is widely used to treat these patients, but long-term quantitative evaluation ofthe effects of liver transplantation on the progression of the neuropathy are not available, We have treated 45 patients with symptomatic TTR-FAP, including 43 with the Met30 TTR gene mutation, and report on the results of periodic evaluation of markers of neuropathy in 25 of them, who have been followed for more than 2 years after liver transplantation (mean follow-up 3 years). The overall survival rates at 1 and 5 years were 82 and 60%, respectively. Urinary incontinence and a low Norris score at liver transplantation were associated with poorer outcome. The motor score stabilized in seven of 11 patients (64%) with mild sensorimotor neuropathy (walking unaided) and in two of the eight patients (25%) with severe sensorimotor deficit (walking with aid) at liver transplantation. In five other patients, deterioration of motor deficit occurred only within the first year after liver transplantation, but was progressive after this interval in two patients. None of the sis patients with pure sensory neuropathy developed motor loss and superficial sensory loss remained unchanged. Two Sears after liver transplantation, the rate of myelinated axon loss in nerve biopsy specimens was markedly lower in seven transplanted patients (0.9/mm(2) of endoneurial area/month) than in non-transplanted patients (70/nm(2) of endoneurial area/month). Symptoms of dysautonomia and quantitated cardiocirculatory autonomic tests remained unchanged. In all patients, serum mutated TTR decreased to 2.5% of pre-liver transplantation values and remained at this level during follow-up. We presently recommend liver transplantation in FAP patients at onset offirst symptoms and exclusion of those with a Norris score below 55 and/or with urinary incontinence.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 14/07/20 alle ore 12:47:35