○種別 (必須): | □ | 学術論文 (審査論文)
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○言語 (必須): | □ | 英語
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○招待 (推奨): |
○審査 (推奨): | □ | Peer Review
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○カテゴリ (推奨): | □ | 研究
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○共著種別 (推奨): |
○学究種別 (推奨): |
○組織 (推奨): |
○著者 (必須): | 1. | 坂下 直実
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| 2. | 香川 聖子
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| 3. | (英) Date Risako (日) (読)
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| 4. | (英) Ueno Tatsuro (日) (読)
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| 5. | (英) Nakagawa Takenobu (日) (読)
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| 6. | (英) Yamashita Taro (日) (読)
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| 7. | (英) Obayashi Konen (日) (読)
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| 8. | (英) Ohshima Toshinori (日) (読)
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| 9. | (英) Ueda Mitsuharu (日) (読)
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| 10. | 堀口 英久
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| 11. | (英) Ando Yukio (日) (読)
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| 12. | (英) Takeya Motohiro (日) (読)
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○題名 (必須): | □ | (英) Marked cardiomegaly in a patient with familial amyloidotic polyneuropathy after orthotopic liver transplantation: a case study. (日)
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○副題 (任意): |
○要約 (任意): | □ | (英) Hepatocyte-derived mutant amyloidogenic transthyretin (ATTR) causes familial amyloidotic polyneuropathy (FAP), for which orthotopic liver transplantation is an established curative treatment. However, some patients with FAP have cardiac amyloidosis after transplantation. Here, we describe a man with an autonomic disorder diagnosed as FAP ATTR Val30Met and marked cardiomegaly after liver transplantation. He underwent orthotopic liver transplantation at 49 years of age and was prescribed prednisolone to prevent graft rejection. Two years later, autonomic dysfunction and severe heart failure gradually developed. He died suddenly at 59. The autopsy revealed marked cardiomegaly (heart weight: 1020 g). Histological and ultrastructural examinations demonstrated massive amyloid deposition and unusual myocardial hypertrophic injury associated with nuclear translocation of the glucocorticoid receptor (GR). No other FAP patients without heart failure showed GR nuclear translocation. GR is a nuclear transcription factor that leads to myocardial hypertrophy, and cumulative prednisolone doses may promote marked cardiomegaly and severe cardiac amyloidosis. (日)
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○キーワード (推奨): | 1. | (英) Amino Acid Substitution (日) (読)
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| 2. | (英) Amyloid (日) (読)
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| 3. | (英) Amyloid Neuropathies, Familial (日) (読)
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| 4. | (英) Amyloidosis (日) (読)
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| 5. | (英) Autopsy (日) (読)
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| 6. | (英) Cardiomegaly (日) (読)
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| 7. | (英) Fatal Outcome (日) (読)
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| 8. | (英) Glucocorticoids (日) (読)
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| 9. | (英) Graft Rejection (日) (読)
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| 10. | 心不全 (heart failure)
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| 11. | (英) Humans (日) (読)
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| 12. | (英) Liver Transplantation (日) (読)
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| 13. | (英) Male (日) (読)
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| 14. | (英) Middle Aged (日) (読)
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| 15. | (英) Mutation (日) (読)
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| 16. | 心筋 (myocardium)
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| 17. | (英) Polyneuropathies (日) (読)
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| 18. | (英) Prednisolone (日) (読)
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○発行所 (推奨): |
○誌名 (必須): | □ | Pathology International ([社団法人 日本病理学会])
(pISSN: 1320-5463, eISSN: 1440-1827)
○ISSN (任意): | □ | 1440-1827
ISSN: 1320-5463
(pISSN: 1320-5463, eISSN: 1440-1827) Title: Pathology internationalTitle(ISO): Pathol IntSupplier: Japanese Society of PathologyPublisher: Blackwell (NLM Catalog)
(医中誌Web)
(Wiley)
(Scopus)
(CrossRef)
(Scopus information is found. [need login])
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○巻 (必須): | □ | 63
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○号 (必須): | □ | 5
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○頁 (必須): | □ | 260 265
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○都市 (任意): |
○年月日 (必須): | □ | 西暦 2013年 5月 初日 (平成 25年 5月 初日)
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○URL (任意): |
○DOI (任意): | □ | 10.1111/pin.12055 (→Scopusで検索)
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○PMID (任意): | □ | 23714253 (→Scopusで検索)
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○CRID (任意): |
○WOS (任意): | □ | 000319659500004
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○Scopus (任意): | □ | 2-s2.0-84900287882
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○備考 (任意): | 1. | (英) Article.ELocationID: 10.1111/pin.12055 (日)
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| 2. | (英) Article.Affiliation: Department of Cell Pathology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan. naomi@tokushima-u.ac.jp (日)
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| 3. | (英) Article.PublicationTypeList.PublicationType: Journal Article (日)
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