CLINICAL IMPROVEMENT AFTER LIVER-TRANSPLANTATION FOR TYPE-I FAMILIAL AMYLOID POLYNEUROPATHY

Long-term results of 13 liver transplantations in patients with a prev ious diagnosis of type I familial amyloid polyneuropathy (FAP) are pre sented. The diagnosis of type I FAP was based on the presence of a bio chemical marker in the plasma (TTR-Met-30 in 11 patients, TTR-Ala-71 i n two). Maximum follow-up is 28 months and the survival rate stands at 11 and 13 patients. Two patients died from sepsis at 2 and 6 months. TTR disappeared from plasma in all cases. Neurological status improved in all eight patients undergoing transplantation more than 6 months p reviously, although electromyographic studies showed a slight improvem ent only in the six with follow-up of more than 1 year. All 13 patient s showed a hyperdynamic haemodynamic pattern with a high incidence (fo ur patients) of the use of venovenous bypass due to haemodynamic intol erance. Two patients also received transplants by the 'piggy-back' tec hnique. In conclusion, liver transplantation may be useful in the trea tment of certain patients with FAP to halt and improve the neurologica l consequences of the disease.