Takei Yo-ichi, Gono Takahisa, Yazaki Masahide, Ikeda Shu-ichi, Ikegami Toshihiko, Hashikura Yasuhiko, Miyagawa Shin-ichi, Hoshii Yoshinobu
Department of Internal Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
Liver Transpl. 2007 Feb;13(2):215-8. doi: 10.1002/lt.20954.
Familial amyloid polyneuropathy (FAP) is a form of hereditary generalized amyloidosis. Liver tissue explanted from FAP patients has normal structure and function, except for the production of amyloidogenic variant transthyretin (TTR), and domino liver transplantation (DLT) using grafts from FAP patients was first performed in 1995. FAP symptoms usually develop in genetically determined individuals after the age of 20, but it is difficult to estimate when FAP symptoms will appear in domino recipients. Concerning this problem, histological findings showing amyloid deposition have recently been obtained in a few domino recipients of FAP livers. This study investigated the presence of de novo amyloid deposition in the gastroduodenal mucosa of domino recipients transplanted at our institution. Biopsy of gastroduodenal mucosa was carried out in 5 recipients of FAP livers and TTR-derived amyloid deposits were detected in 2 patients, both of whom had undergone DLT 47 months previously. In FAP liver recipients, de novo systemic amyloid deposition may begin much sooner than previously supposed. Therefore, careful follow-up of domino recipients of FAP livers is required.
家族性淀粉样多神经病（FAP）是遗传性全身性淀粉样变性的一种形式。从FAP患者身上取出的肝脏组织结构和功能正常，但会产生淀粉样前体蛋白变体转甲状腺素蛋白（TTR），1995年首次进行了使用FAP患者肝脏移植物的多米诺肝移植（DLT）。FAP症状通常在20岁以后在基因决定的个体中出现，但很难估计FAP症状会在多米诺肝移植受者中何时出现。关于这个问题，最近在一些接受FAP肝脏移植的多米诺受者中获得了显示淀粉样蛋白沉积的组织学结果。本研究调查了在我们机构接受移植的多米诺受者胃十二指肠黏膜中是否存在新生淀粉样蛋白沉积。对5名接受FAP肝脏移植的受者进行了胃十二指肠黏膜活检，在2名患者中检测到TTR源性淀粉样蛋白沉积，这两名患者均在47个月前接受了DLT。在FAP肝脏移植受者中，新生全身性淀粉样蛋白沉积可能比以前认为的要早得多开始。因此，需要对接受FAP肝脏移植的多米诺受者进行仔细的随访。
