Liver transplantation in transthyretin familial amyloid polyneuropathy: first report from Argentina

This is the first report from Argentina of liver transplantation in patient s with transthyretin related familial amyloidotic polyneuropathy. The aims of the study were to analyse the clinical characteristics of this new focus and evaluate the postoperative complications and long term follow up. Five of ten patients evaluated underwent liver transplantation. During the wait ing period the polyneuropathy disability score in each patient progressed o ne or two stages. Pretransplant modified body mass index was 723. The proce dure was done with full size grafts in four cases and a split right graft i n one. All patients presented postoperative complications related to diseas e: severe edema of the legs, recurrent choledochal lithiasis, postoperative hernia, necrotizing fasciitis and ischemic rectosigmoidal perforation. Ass essment of three patients after 20 months of transplantation showed improve ment in somatic and mental symptoms. No improvement was noted in cardiac de nervation and gastric stasis. Liver transplantation is a rational therapeut ic option for transthyretin familial amyloidotic polyneuropathy in Argentin a and should be indicated in earlier stages of the symptomatic disease to r educe the postoperative morbidity and mortality. Family studies and follow up of asymptomatic carriers will define the epidemiological behavior in thi s country and facilitate early therapeutic intervention.