{"created":"2023-06-19T07:54:28.896330+00:00","id":818,"links":{},"metadata":{"_buckets":{"deposit":"e3be0235-9a90-46a6-8287-769c5af4266b"},"_deposit":{"created_by":4,"id":"818","owners":[4],"pid":{"revision_id":0,"type":"depid","value":"818"},"status":"published"},"_oai":{"id":"oai:niimi-c.repo.nii.ac.jp:00000818","sets":["31:32:42"]},"author_link":["2152","2154","2150","2153","2151","2149"],"item_5_biblio_info_12":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2008-01-01","bibliographicIssueDateType":"Issued"},"bibliographicPageEnd":"7","bibliographicPageStart":"1","bibliographicVolumeNumber":"29","bibliographic_titles":[{"bibliographic_title":"新見公立短期大学紀要"},{"bibliographic_title":"The bulletin of Niimi College","bibliographic_titleLang":"en"}]}]},"item_5_description_10":{"attribute_name":"抄録(日)","attribute_value_mlt":[{"subitem_description":"Alport症候群は難聴または視力障害を伴う遺伝性の腎疾患で、通常男児が重症となる。原因遺伝子としてIV型コラーゲンのa3鎖またはa4鎖、a5鎖遺伝子の突然変異が明らかにされている。a5鎖遺伝子が原因であるX染色体連鎖優性型が最も頻度が高い。臨床症状、糸球体基底膜の多層化、免疫組織学によるa(IV)鎖の解析、a(IV)鎖の遺伝子解析から診断が可能である。遺伝性腎疾患の中では嚢胞性腎疾患に続いて患者数が多いとされているが、国内での患者数や発生頻度に関する研究は不十分である。本総説では、これまでに公表されている国内外の文献を検討し、日本国内のAlport症候群患者数の推定を試みた。比較的最近の報告によると、スウェーデン南部の男性で4万人あたり一人の患者数となる。この頻度から日本の患者数を推定すると、19歳以下の男性に限っても300人の患者数となる。これまで国内の患者数について報告された数よりかなり多い数である。更なる調査の必要性が示唆された。","subitem_description_type":"Other"}]},"item_5_description_11":{"attribute_name":"抄録(英)","attribute_value_mlt":[{"subitem_description":"Alport syndrome is a hereditary renal disease accompanied with ocular abnormality or hearing loss. The disease is diagnosed with clinical features, abnormality of glomerular basement membrane, loss of alpha 3 to alpha 5 chains of collagen IV, and/or a mutation of collagen IV genes. The occurrence of the disease has been studied in several reports, but that of Japanese Alport patients has not been fully investigated. The aim of this review is to evaluate the previously published papers, and to estimate the number or the incidence of the patients in Japan. Based on the report of Persson et al. (2006), in which the prevalence of the disease was demonstrated to be one in 40,000 in males, the number of the Japanese male patients (～19 years) will be calculated to be 300. But this number is different from those in the previous reports. Our review indicates the necessity of the further investigation of the disease.","subitem_description_type":"Other"}]},"item_5_description_15":{"attribute_name":"表示順","attribute_value_mlt":[{"subitem_description":"3","subitem_description_type":"Other"}]},"item_5_description_16":{"attribute_name":"アクセション番号","attribute_value_mlt":[{"subitem_description":"KJ00005818577","subitem_description_type":"Other"}]},"item_5_description_8":{"attribute_name":"記事種別(日)","attribute_value_mlt":[{"subitem_description":"総説","subitem_description_type":"Other"}]},"item_5_identifier_registration":{"attribute_name":"ID登録","attribute_value_mlt":[{"subitem_identifier_reg_text":"10.51074/00000802","subitem_identifier_reg_type":"JaLC"}]},"item_5_source_id_1":{"attribute_name":"雑誌書誌ID","attribute_value_mlt":[{"subitem_source_identifier":"AA11439373","subitem_source_identifier_type":"NCID"}]},"item_5_source_id_19":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"13453599","subitem_source_identifier_type":"ISSN"}]},"item_5_text_6":{"attribute_name":"著者所属(日)","attribute_value_mlt":[{"subitem_text_value":"新見公立短期大学看護学科"},{"subitem_text_value":"新見公立短期大学幼児教育学科"},{"subitem_text_value":"三重大学医学部第一内科:三重大学医学部附属病院血液浄化療法部"}]},"item_5_text_7":{"attribute_name":"著者所属(英)","attribute_value_mlt":[{"subitem_text_language":"en","subitem_text_value":"Department of Nursing, Niimi College"},{"subitem_text_language":"en","subitem_text_value":"Department of Early Childhood Education, Niimi College"},{"subitem_text_language":"en","subitem_text_value":"Department of First Internal Medicine, Mie University Medical School"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"内藤, 一郎"},{"creatorName":"ナイトウ, イチロウ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{"nameIdentifier":"2149","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"斎藤, 健司"},{"creatorName":"サイトウ, ケンジ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{"nameIdentifier":"2150","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"[ノ]村, 信介"},{"creatorName":"ノムラ, シンスケ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{"nameIdentifier":"2151","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"NAITO, Ichiro","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"2152","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"SAITO, Kenji","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"2153","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"NOMURA, Shinsuke","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"2154","nameIdentifierScheme":"WEKO"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2017-05-16"}],"displaytype":"detail","filename":"KJ00005818577.pdf","filesize":[{"value":"836.7 kB"}],"format":"application/pdf","licensetype":"license_11","mimetype":"application/pdf","url":{"label":"KJ00005818577.pdf","url":"https://niimi-c.repo.nii.ac.jp/record/818/files/KJ00005818577.pdf"},"version_id":"c6a37e8e-b549-4c60-8476-e36c578148fc"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Alport症候群","subitem_subject_scheme":"Other"},{"subitem_subject":"IV型コラーゲン","subitem_subject_scheme":"Other"},{"subitem_subject":"遺伝性腎症","subitem_subject_scheme":"Other"},{"subitem_subject":"頻度","subitem_subject_scheme":"Other"},{"subitem_subject":"疫学","subitem_subject_scheme":"Other"},{"subitem_subject":"Alport syndrome","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"collagen type IV","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"hereditary nephritis","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"frequency","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"incidence","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"epidemiology","subitem_subject_language":"en","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"jpn"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"departmental bulletin paper","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"Alport症候群の発生頻度","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Alport症候群の発生頻度"},{"subitem_title":"Incidence of Alport Syndrome Patients","subitem_title_language":"en"}]},"item_type_id":"5","owner":"4","path":["42"],"pubdate":{"attribute_name":"公開日","attribute_value":"2017-05-16"},"publish_date":"2017-05-16","publish_status":"0","recid":"818","relation_version_is_last":true,"title":["Alport症候群の発生頻度"],"weko_creator_id":"4","weko_shared_id":-1},"updated":"2023-06-19T08:06:25.424314+00:00"}