PARTIAL LIVER-TRANSPLANTATION FROM LIVING DONORS IN FAMILIAL AMYLOID POLYNEUROPATHY

Partial liver transplantation using a graft from a living donor (PLT) has been carried out for children with incurable hepatic disorders. We have extended this technique to 5 adult patients (4 female and 1 male ) with familial amyloid polyneuropathy (FAP). All were proven to have a TTR Met30 mutation. Four of these 5 patients are alive 5 to 28 month s after operation, and 3 patients with a shorter duration of illness ( less than five years) showed remarkable clinical improvement. Periodic nausea and vomiting ceased and other autonomic symptoms including ort hostatic hypotension, abnormal bowel movements and dysuria were also i mproving soon after PLT. Somatic motor and sensory neuropathic symptom s tended to improve very gradually. On the other hand, further progres sion of FAP symptoms was observed in one severely disabled patient wit h a 7 year-history of this disease. One other patient with a similar c ondition died 3 months after PLT. The donors who consisted of 2 sister s and 3 husbands recovered without complications and returned to their previous social lives 2 months after operation. Recently, it has been shown that liver transplantation corrects the metabolic abnormality i n FAP and therefore liver transplantation has been widely accepted as a treatment for FAP. Since cadaveric donors are seldom available in Ja pan, we started to perform PLT for FAP patients and have demonstrated good results. PLT can safely be done on adult patients and this treatm ent seems to be more effective for FAP patients at an early stage.