[Liver transplantation in familial amyloidosis]

A 32-year-old Portuguese with hereditary amyloidosis had been suffering from polyneuropathy for 9 years. It began insidiously with polyneuropathic complaints in the legs which gradually got worse over the years and progressively impaired walking. He also had signs of autonomic neuropathy with severe orthostatic dysregulation, abnormal micturition and impotence. His general state had deteriorated during the last 3 years with a weight loss of 18 kg, due to treatment-resistant diarrhoea. As there is so far no known cure of the amyloidosis, which usually ends fatally from cachexia after an average of 10 years, liver transplantation was performed to reduce amyloid production and thus favourably influence the course of the disease. The patient's general condition has remained stable 32 months after the transplantation.