title: Transthyretin-derived amyloid deposition on the gastric mucosa in domino recipients of familial amyloid polyneuropathy liver.
abstract: 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.
authors.affiliation: Department of Internal Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan. y-takei@hsp.md.shinshu-u.ac.jp
authors.forename: Yo-ichi
authors.initials: Y
authors.lastname: Takei
authors.forename: Takahisa
authors.initials: T
authors.lastname: Gono
authors.forename: Masahide
authors.initials: M
authors.lastname: Yazaki
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Ikegami
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Hashikura
authors.forename: Shin-ichi
authors.initials: S
authors.lastname: Miyagawa
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Hoshii
mesh_terms: D000328:Adult; D000682:Amyloid / Q000032:analysis*; D028227:Amyloid Neuropathies, Familial / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D005753:Gastric Mucosa / Q000737:chemistry* / Q000473:pathology*; D006801:Humans; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D011228:Prealbumin / Q000378:metabolism; D009927:Tissue and Organ Procurement / Q000379:methods*
chemical_list: D000682:Amyloid; D011228:Prealbumin