Long-term results of liver transplantation in familial amyloidotic polyneuropathy type I.
BACKGROUND
The aim of this article is to report our experience regarding the survival and the evolution of polyneuropathy of the extremities and autonomic dysfunction in 18 liver transplant patients with familial amyloidotic polyneuropathy type I after a mean follow-up of more than 2.5 years for 13 patients.


METHODS
The actuarial survival rate of the 18 patients is 72.2% and 60.1%, respectively, at 12 and 58 months.


RESULTS
In all the patients we noted clinical improvement of the polyneuropathy of the extremities and autonomic dysfunction during the first 6 months after transplant. The clinical data due to autonomic nervous system involvement showed an earlier improvement than those due to nervous motor involvement.


CONCLUSIONS
In conclusion, our results suggest that liver transplant may be useful in the treatment of certain cases of familial amyloidotic polyneuropathy to stop the neurological deterioration of the patients and to avoid the fatal end of the disease.
Parrilla
P
P
Department of Surgery, Virgen de la Arrixaca University Hospital, Murcia, Spain.
Ramirez
P
P
Andreu
L F
LF
Bueno
S F
SF
Robles
R
R
Miras
M
M
Acosta
F
F
Viader-Farre
D
D
de Mingo
P
P
Bermejo
J
J
Garrido
S F
SF
Munar-Ques
M
M
D000328:Adult; D017772:Amyloid Neuropathies / Q000503:physiopathology / Q000601:surgery*; D004576:Electromyography; D005500:Follow-Up Studies; D006801:Humans; D016031:Liver Transplantation / Q000401:mortality; D008875:Middle Aged; D015996:Survival Rate