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

1. 免疫抑制剂对大鼠甲状腺转素（TTR）的影响。服用他克莫司4周使TTR降低8%，而环孢素没有。这一发现将有助于移植后家族性淀粉样蛋白多发性神经病（FAP）患者和多米诺骨牌受体的免疫抑制剂的选择。用带有人变异ttr基因的转基因大鼠肝脏建立多米诺骨牌肝移植动物模型：移植的大鼠成活率不佳，但从1周到3个月共有3只大鼠存活。有趣的是，异常的TTR水平低于多米诺骨牌“供体”（原转基因大鼠）。这一现象的原因尚未解决，应该进一步调查，因为它可能提供一种方法来降低多米诺骨牌受体发病的风险。FAP患者和骨牌接受者移植后随访：连续34例FAP患者移植后10年生存率为100%。所有患者在移植后均未出现神经系统症状的恶化，但有9例出现心脏异常，4例出现眼部症状的进展。92%的患者可以回到全职工作岗位。在8名接受多米诺骨牌治疗的患者中，有2人死于肝癌复发，但另外6人可以恢复日常生活。在2 - 5年的随访中，胃肠道粘膜未见淀粉样蛋白沉积。应继续随访，监测FAP患者和多米诺骨牌受体的临床状况。

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.