TREATMENT OF A JAPANESE PATIENT WITH FAMILIAL AMYLOIDOTIC POLYNEUROPATHY WITH ORTHOTOPIC LIVER-TRANSPLANTATION

A 28-year-old male patient with familial amyloidotic polyneuropathy (F AP) underwent a liver transplantation from a heart-beating cadaveric d onor in Sweden. He had suffered from the disease for 2.5 years. It too k 5.5 hours to carry out the operation without blood transfusion. Afte r the liver transplantation, serum amyloidgenic variant transthyretin (TTR) levels became extremely low and dirrhea stopped after the 7th da y. On day 13, the patient was discharged from the hospital and one mon th after the transplantation, his general condition remained quite goo d. This is the first case of a Japanese patient with congenital metabo lic disorders as well as FAP to receive a liver transplantation from a heart-beating cadaveric donor.