Research for the factors that Will influence the onset of the oriainal disease in the recipients who underwent a sequential (domino) iver transplantation with the use offamilial amyloid polyneuropahy liver

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1. Effect of immunosuppressants on the transthyretin (TTR) in rats. : Administration of tacrolimus for 4 weeks decreased the TTR in 8%, although cyclosporine did not. This finding will be useful for the selection of immunosuppressants in post-transplant familial amyloid polyneuropathy (FAP) patient and domino recipients.2. Animal model of domino liver transplantation using a liver taken from a transgenic rat with a human variant-TTR gene : Survival rate of the domino-transplanted rat was not good, but totally 3 rats survived from 1 week to 3 months. Interestingly, aberrant TTR level was lower than that of the domino "donor" (the original transgenic rat). The cause of this phenomenon remained unresolved, and should be further investigated because it may offer a way to decrease the risk of the onset of the original disease in domino recipients.3. Post-transplant follow up of FAP patients and domino recipients : Post-transplant ten year survival of consecutive 34 FAP patients was 100%. Any showed no deterioration of the neurological symptoms after the transplantation, although 9 showed cardiac abnormalities, and four showed progression of ocular symptoms. Ninety-two percent of the patients could return to be a full-time worker. In eight domino recipients, two died of the recurrence of the liver cancer, but the other 6 could return to their full-time daily activity. In the 2 - 5 years of follow-up, there has been no deposit of the amyloid in the mucosa of the gastrointestinal tract. Follow up should be continued to monitor the clinical status of the FAP patients and domino recipients.