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本邦における多発性内分泌腺腫瘍症1型の実態調査
https://tokushima-u.repo.nii.ac.jp/records/2004871
https://tokushima-u.repo.nii.ac.jp/records/2004871403973f7-d742-4131-99a1-6ed110dec4ac
名前 / ファイル | ライセンス | アクション |
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Item type | 文献 / Documents(1) | |||||||||||||||||
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公開日 | 2018-05-31 | |||||||||||||||||
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アクセス権 | open access | |||||||||||||||||
資源タイプ | ||||||||||||||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||||||||||||||
資源タイプ | journal article | |||||||||||||||||
出版社版DOI | ||||||||||||||||||
識別子タイプ | DOI | |||||||||||||||||
関連識別子 | https://doi.org/10.1507/endocrine1927.67.7_764 | |||||||||||||||||
言語 | ja | |||||||||||||||||
関連名称 | 10.1507/endocrine1927.67.7_764 | |||||||||||||||||
出版タイプ | ||||||||||||||||||
出版タイプ | VoR | |||||||||||||||||
出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||||||||||||||
タイトル | ||||||||||||||||||
タイトル | 本邦における多発性内分泌腺腫瘍症1型の実態調査 | |||||||||||||||||
言語 | ja | |||||||||||||||||
タイトル別表記 | ||||||||||||||||||
その他のタイトル | Clinical Characteristics in Multiple Endocrine Neoplasia Type 1 in Japan : A review of 106 patients | |||||||||||||||||
言語 | en | |||||||||||||||||
著者 |
吉本, 勝彦
× 吉本, 勝彦
WEKO
1648
× 斎藤, 史郎
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抄録 | ||||||||||||||||||
内容記述タイプ | Abstract | |||||||||||||||||
内容記述 | A review of 106 patients with multiple endocrine neoplasia (MEN) type 1 reported between 1966-1989 in Japan was conducted in order to clarify the natural history of this disease. Sporadic MEN 1 was found in 61 patients, and familial MEN 1 was found in 45 patients from 15 families. The mean ages at diagnosis of the two groups were 46.2 and 41.3 years, respectively, and the male to female ratio was 3:4. With regards to the involvement of the pituitary, parathyroid and endocrine pancreas, the combination of three endocrine glands was 31%, and that of two was 48%, in which the pituitary, parathyroid and endocrine pancreas had tumorous lesions in 60%, 88% and 63%, respectively. The first clinical manifestations of MEN 1 were the symptoms of hyperparathyroidism (32%), pituitary tumors (26%), peptic ulcer (28%) and hypoglycemia (13%). These symptoms appeared between 6 and 57 years of age (mean 34 years). The main clinical symptoms of the pituitary tumors were acromegaly and gigantism (37%), galactorrhea-amenorrhea syndrome (20%), Cushing's disease (10%) and visual disturbance due to compression of the tumor (20%). The clinical manifestations of hyperparathyroidism were mainly asymptomatic hypercalcemia (41%), nephrolithiasis (42%) and osteitis fibrosa (5%). In the patients with pancreatic tumor, Zollinger-Ellison syndrome (52%) and hypoglycemic symptoms (42%) were found. Tumors in the adrenal cortex, thyroid, carcinoid and lipoma were detected in association with MEN 1 in the frequencies of 28%, 21%, 9% and 5%, respectively. Death was reported in 37 patients between 9 and 86 years of age (mean 50.0 years). The cause of death was gastrointestinal bleeding and perforation (45%), surgery (24%) and disseminated carcinomatosis (18%). Recently, the gene predisposing to this syndrome has been assigned to chromosome 11 (11q13) in non-Japanese cases but not yet in Japanese MEN 1 patients. |
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言語 | en | |||||||||||||||||
書誌情報 |
ja : 日本内分泌学会雑誌 en : Folia Endocrinologica Japonica 巻 67, 号 7, p. 764-774, 発行日 1991 |
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収録物ID | ||||||||||||||||||
収録物識別子タイプ | ISSN | |||||||||||||||||
収録物識別子 | 2186506X | |||||||||||||||||
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収録物識別子タイプ | ISSN | |||||||||||||||||
収録物識別子 | 00290661 | |||||||||||||||||
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収録物識別子タイプ | NCID | |||||||||||||||||
収録物識別子 | AN00195891 | |||||||||||||||||
出版者 | ||||||||||||||||||
出版者 | 一般社団法人 日本内分泌学会 | |||||||||||||||||
言語 | ja | |||||||||||||||||
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識別子 | 71582 | |||||||||||||||||
識別子タイプ | URI | |||||||||||||||||
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言語 | jpn |