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Research paper
Changes in pathological and biochemical findings of systemic tissue sites in familial amyloid polyneuropathy more than 10 years after liver transplantation
  1. Toshinori Oshima1,
  2. Satomi Kawahara2,
  3. Mitsuharu Ueda1,
  4. Yushi Kawakami2,
  5. Rina Tanaka2,
  6. Takahiro Okazaki1,
  7. Yohei Misumi1,
  8. Konen Obayashi2,
  9. Taro Yamashita1,
  10. Yuki Ohya3,
  11. Elisabet Ihse4,
  12. Satoru Shinriki2,
  13. Masayoshi Tasaki1,
  14. Hirofumi Jono2,
  15. Katsuhiro Asonuma3,
  16. Yukihiro Inomata3,
  17. Per Westermark4,
  18. Yukio Ando1
  1. 1Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Kumamoto, Japan
  2. 2Department of Diagnostic Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Kumamoto, Japan
  3. 3Department of Transplantation and Pediatric Surgery, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Kumamoto, Japan
  4. 4Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden
  1. Correspondence to Dr Mitsuharu Ueda, Department of Neurology, Graduate School Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan; mitt{at}rb3.so-net.ne.jp

Abstract

Objective To elucidate the long-term effects of liver transplantation (LT) on familial amyloid polyneuropathy (FAP).

Methods We investigated clinicopathological and biochemical characteristics of systemic tissues in four autopsied cases of FAP patients surviving more than 10 years after LT and seven autopsied cases without LT. For analysing the truncated form of transthyretin (TTR) in amyloid, we also employed specimens from additional 18 FAP patients.

Results Several tissue sites such as the heart, tongue and spinal cord had moderate-to-severe amyloid deposits but other tissues showed no or mild amyloid deposition. Those findings seemed similar to those observed in senile systemic amyloidosis (SSA), a sporadic amyloidosis caused by wild-type (WT) TTR. Also, amyloid deposits in systemic tissue sites except for the spinal cord in patients after LT derived mostly from WT TTR secreted from the normal liver grafts. In addition, in non-transplantation patients, proportions of WT TTR seemed to be relatively high in those tissue sites in which patients after LT had severe amyloid deposition, which suggests that WT TTR tends to form amyloid in those tissue sites. Finally, although the truncation of TTR in amyloid deposits did not depend on undergoing LT, we elucidated the truncation of TTR occurred predominantly in patients from non-endemic areas of Japan, where FAP amyloidogenic TTR V30M patients are late onset and low penetrance, compared with patients from an endemic area of Japan.

Conclusions FAP may shift to systemic WT TTR amyloid formation after LT, which seems to be similar to the process in SSA. The truncation of TTR in amyloid deposits may depend on some genetic or environmental factors other than undergoing LT.

  • Amyloid
  • Genetics
  • Pathology

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