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Transthyretin‐derived amyloid deposition on the gastric mucosa in domino recipients of familial amyloid polyneuropathy liver
Author(s) -
Takei Yoichi,
Gono Takahisa,
Yazaki Masahide,
Ikeda Shuichi,
Ikegami Toshihiko,
Hashikura Yasuhiko,
Miyagawa Shinichi,
Hoshii Yoshinobu
Publication year - 2007
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.20954
Subject(s) - transthyretin , medicine , liver transplantation , amyloidosis , amyloid (mycology) , amyloid polyneuropathy , pathology , polyneuropathy , transplantation , domino , gastroenterology , disease , age of onset , biology , biochemistry , catalysis
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. Liver Transpl, 2006. © 2006 AASLD.