WORLDWIDE SURVEY OF LIVER-TRANSPLANTATION IN PATIENTS WITH FAMILIAL AMYLOIDOTIC POLYNEUROPATHY

The First International Workshop on liver transplantation in patients with familial amyloid polyneuropathy (FAP) was held at Huddinge Hospit al in Stockholm, Sweden, September 10-11, 1993. Information was presen ted on 64 fiver transplant recipients treated at 15 centers in 10 coun tries. Twenty-two patients had a follow-up time of more than 12 months , the longest follow-up was 41 months. Fifty-three patients had surviv ed the procedure, while 11 had died within 8 months after the operatio n. The 1 and 2 year actuarial patient survival rate was 67 per cent. I n most of the 22 patients surviving more than I year; the neurological symptoms had stabilized and the beneficial effect was most apparent a s regards symptoms from the autonomic nervous system. There was consen sus among the participants at the Workshop that liver transplantation for FAP is a worth while procedure and that such transplantation activ ities should continue. Transplantation should preferably be performed before the symptoms become severe and while the nutritional state is s till satisfactory. Early onset of rapid progress of symptoms, in the p atient or among family members, speaks in favor of transplantation.