| ○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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| ○著者 (必須): | 1. | (英) Moriuchi Tsuyoshi (日) (読)
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| 2. | 香川 典子
| ○役割 (任意): | □ | (英) (日) 登録票の集計，考察
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| 3. | (英) Mukoyama Masakuni (日) (読)
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| 4. | (英) Hizawa Kazuo (日) (読)
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| ○題名 (必須): | □ | (英) Autopsy analysis of the muscular dystrophies (日)
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| ○要約 (任意): | □ | (英) Life span, causes of death, weight of heart, liver, brain, and main pathological changes of internal organs were analysed on 329 autopsy cases of muscular dystrophies. These included 249 cases of Duchenne muscular dystrophy (DMD), 3 Becker muscular dystrophies (BMD), 14 limb-girdle muscular dystrophies (LGMD), 3 fascioscapulohumeral muscular dystrophies (FSH), 18 Fukuyama type congenital muscular dystrophies (FCMD) and 17 myotonic dystrophies (MyD). In DMD the life span has definitely prolonged in recent years. Pulmonary infection, which was once the major cause of death, has greatly decreased in recent years. Instead, respiratory and cardiac failures caused by dystrophic changes of respiratory and cardiac muscles were more closely related to the causes of death in many recent cases. Myocardial fibrosis was observed in most of the patients with DMD, BMD, LGMD, FCMD and MyD. The distribution of cardiac lesions was similar in BMD, LGMD and FCMD as in DMD. In MyD the disorders involved more frequently conductive muscles resulting in arrhythmias. The dystrophic cardiomyopathy seemed to be a part of the essential changes in all types of muscular dystrophy, although different in intensity and rate of morbidity. Alzheimer's neurofibrillary changes were observed in the brain of some cases of FCMD and MyD, suggesting the possibility of precocious aging of the brain in some patients of the muscular dystrophies. (日) 329例の筋ジストロフィー剖検登録票をもとに，寿命，死因，心·肝·脳の病理学的所見について調べた．DMDの寿命は近年著しく延長していた．かつては肺炎による死亡が多かったが，最近では減少して心不全や呼吸不全による死亡の割合が増加していた．心筋の繊維化はDMD，BMD，LGMD，FCMD，MyDのほとんどの例で認められた．MyDでは刺激伝導系に繊維化をきたして不整脈の原因となることが多かった．アルツハイマーの神経原生変化はFCMDとMyDの数例にみられたが，脳の早熟性の老化の可能性を示唆しているのかもしれない．
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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. | 脳 (brain)
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| 4. | (英) Cause of Death (日) (読)
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| 5. | 子ども (children)
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| 6. | 女性 (female)
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| 7. | (英) Humans (日) (読)
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| 8. | (英) Liver (日) (読)
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| 9. | (英) Longevity (日) (読)
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| 10. | 男性 (male)
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| 11. | (英) Muscular Dystrophies (日) (読)
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| 12. | 心筋 (myocardium)
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| ○発行所 (推奨): | □ | 徳島大学.医学部
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| ○誌名 (必須): | □ | The Tokushima Journal of Experimental Medicine ([徳島大学.医学部])
(pISSN: 0040-8875)
| ○ISSN (任意): | □ | 0040-8875
ISSN: 0040-8875
(pISSN: 0040-8875) Title: The Tokushima journal of experimental medicineTitle(ISO): Tokushima J Exp MedSupplier: 徳島大学Publisher: Tokushima University School of Medicine (NLM Catalog)
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| ○巻 (必須): | □ | 40
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| ○号 (必須): | □ | 1-2
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| ○頁 (必須): | □ | 83 93
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| ○都市 (任意): |
| ○年月日 (必須): | □ | 西暦 1993年 3月 初日 (平成 5年 3月 初日)
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| ○URL (任意): |
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| ○PMID (任意): | □ | 8211986 (→Scopusで検索)
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| ○備考 (任意): | 1. | (英) Article.PublicationTypeList.PublicationType: Journal Article (日)
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