LONG-TERM RESULTS OF LIVER-TRANSPLANTATION IN FAMILIAL AMYLOIDOTIC POLYNEUROPATHY TYPE-I

Background. The aim of this article is to report our experience regard ing the survival and the evolution of polyneuropathy of the extremitie s and autonomic dysfunction in 18 liver transplant patients with famil ial amyloidotic polyneuropathy type I after a mean follow-up of more t han 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 po lyneuropathy of the extremities and autonomic dysfunction during the f irst 6 months after transplant. The clinical data due to autonomic ner vous system involvement showed an earlier improvement than those due t o nervous motor involvement. Conclusions. In conclusion, our results s uggest 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 disea se.