○種別 (必須): |
○言語 (必須): | □ | 英語
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○招待 (推奨): |
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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. | (英) Takahashi Kiyoshi (日) (読)
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| 2. | 坂下 直実
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| 3. | (英) Ando Yukio (日) (読)
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| 4. | (英) Suga Moritaka (日) (読)
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| 5. | (英) Ando Masayuki (日) (読)
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○題名 (必須): | □ | (英) Late onset type I familial amyloidotic polyneuropathy: presentation of three autopsy cases in comparison with 19 autopsy cases of the ordinary type. (日)
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○要約 (任意): | □ | (英) Clinicopathological features of three autopsy cases of extremely rare late onset type I familial amyloidotic polyneuropathy were presented and compared with 19 autopsy cases of the ordinary type. In the late onset cases, the ages at onset and at death were 27.5 and 24.5 years older, respectively, compared with the ordinary type. Also, duration of the total clinical course from onset to death was 3.7 years less than in the late onset cases. The degree of amyloid deposition was more marked in the heart of the late onset cases, causing prominent cardiac hypertrophy. It was also marked in the kidneys or thyroid of two cases, but slight to moderate in the peripheral or autonomic nervous tissues in all cases. Immunohistochemical investigation demonstrated the presence of transthyretin (TTR) as an amyloid precursor protein and of serum amyloid P-component in amyloid deposits in various organs and tissues of the late onset type. These findings, as well as serum levels of variant TTR, were similar to those of the ordinary type. These results suggest that there are some factors other than the amyloid precursor protein that effect the degree of amyloid deposition. (日)
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○キーワード (推奨): | 1. | (英) Adolescent (日) (読)
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| 2. | (英) Adult (日) (読)
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| 3. | (英) Age of Onset (日) (読)
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| 4. | (英) Aged (日) (読)
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| 5. | (英) Amyloid (日) (読)
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| 6. | (英) Amyloid Neuropathies (日) (読)
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| 7. | 女性 (female)
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| 8. | (英) Histocytochemistry (日) (読)
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| 9. | (英) Humans (日) (読)
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| 10. | (英) Kidney (日) (読)
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| 11. | 男性 (male)
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| 12. | (英) Middle Aged (日) (読)
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| 13. | 心筋 (myocardium)
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| 14. | (英) Nerve Fibers (日) (読)
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| 15. | (英) Pedigree (日) (読)
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| 16. | (英) Tissue Distribution (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): | □ | 1320-5463
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): | □ | 47
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○号 (必須): | □ | 6
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○頁 (必須): | □ | 353 359
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○都市 (任意): |
○年月日 (必須): | □ | 西暦 1997年 6月 初日 (平成 9年 6月 初日)
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○URL (任意): |
○DOI (任意): | □ | 10.1111/j.1440-1827.1997.tb04508.x (→Scopusで検索)
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○PMID (任意): | □ | 9211522 (→Scopusで検索)
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○CRID (任意): |
○WOS (任意): |
○Scopus (任意): | □ | 2-s2.0-0030942753
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○備考 (任意): | 1. | (英) Article.Affiliation: Second Department of Pathology, Kumamoto University School of Medicine, Japan. (日)
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| 2. | (英) Article.PublicationTypeList.PublicationType: Case Reports (日)
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| 3. | (英) Article.PublicationTypeList.PublicationType: Comparative Study (日)
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| 4. | (英) Article.PublicationTypeList.PublicationType: Journal Article (日)
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