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素运载蛋白（TTR）的影响。：他克莫司给药4周使TTR降低8%，但环孢素没有。这一发现将有助于移植后家族性淀粉样多发性神经病（FAP）患者和多米诺骨牌效应者免疫抑制剂的选择.采用转人TTR基因大鼠肝脏的多米诺骨牌肝移植动物模型：多米诺骨牌移植大鼠存活率不高，但共有3只大鼠存活1周至3个月。有趣的是，异常TTR水平低于多米诺骨牌“供体”（原始转基因大鼠）。这一现象的原因尚未解决，应进一步调查，因为它可能提供一种方法来降低多米诺骨牌原始疾病的发病风险。FAP患者和多米诺受体移植后随访：连续34例FAP患者移植后10年生存率为100%。所有患者在移植后没有表现出神经系统症状的恶化，尽管9人表现出心脏异常，4人表现出眼部症状的进展。百分之九十二的病人可以回来做全职工作。在8名多米诺骨牌接受者中，2人死于肝癌复发，但其他6人可以恢复全职日常活动。随访2 ~ 5年，胃肠道粘膜未见淀粉样蛋白存款。应继续随访以监测FAP患者和多米诺骨牌受体的临床状态。

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.