Improvement in the polyneuropathy associated with familial amyloid polyneuropathy after liver transplantation.
Neurology
; 47(4): 944-51, 1996 Oct.
Article
em En
| MEDLINE
| ID: mdl-8857724
ABSTRACT
OBJECTIVE:
To study, following liver transplantation, the neurologic progression or regression of the polyneuropathy in a cohort of patients with familial amyloidotic polyneuropathy (FAP).BACKGROUND:
FAP is characterized by the relentless progression of neurologic and cardiac impairment, leading to death within 7 to 15 years after disease onset. No effective treatment to slow or halt the progression of this disease has been found to date. DESIGN/METHODS:
Over the past 3 years, our FAP patients were offered liver transplantation as treatment. We report on nine patients who were followed longitudinally with serial neurologic examinations since transplantation.RESULTS:
Clinically, all patients evaluated for neurologic progression reported significant improvement in general well being. No patient showed any progression in neurologic disease since receiving a liver transplant. Improvements are documented in symptomatic, autonomic, and sensorimotor neurologic disease in all patients.CONCLUSION:
Our experience suggests that liver transplantation may offer hope for arrest of progression and neurologic improvement in patients with FAP.
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Base de dados:
MEDLINE
Assunto principal:
Transplante de Fígado
/
Neuropatias Amiloides
Tipo de estudo:
Prognostic_studies
/
Risk_factors_studies
Limite:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Ano de publicação:
1996
Tipo de documento:
Article