{"created":"2021-03-01T06:12:42.806667+00:00","id":8682,"links":{},"metadata":{"_buckets":{"deposit":"5ebb4162-2c98-4aee-971c-280591375430"},"_deposit":{"id":"8682","owners":[],"pid":{"revision_id":0,"type":"depid","value":"8682"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00008682","sets":["453:456","471:537:538:909"]},"item_7_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Clinical Significance of Galactose-deficient IgA1 in Familial IgA Nephropathy"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2015-07","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"7","bibliographicPageEnd":"381","bibliographicPageStart":"373","bibliographicVolumeNumber":"129","bibliographic_titles":[{"bibliographic_title":"新潟医学会雑誌"},{"bibliographic_title":"新潟医学会雑誌","bibliographic_titleLang":"en"}]}]},"item_7_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"近年, IgA腎症の発症に糖鎖不全IgA1(Galactose-deficient IgA1:Gd-IgA1)の関与が示唆されている. また家族性IgA腎症患者, およびその血縁者でGd-IgA1が高値であるという報告もあり, IgA1の糖鎖不全に遺伝要因が部分的に関与していることが示唆されている. 我々は日本における家族性IgA腎症家系の収集を行い, 各家系内構成者の糖鎖不全IgA1の測定を行い, 家族性IgA腎症家系におけるGd-IgA1や臨床所見・病理所見との関連を調べた. 腎生検でIgA腎症と診断された患者が複数例存在する家族性IgA腎症の9家系からその構成者34名と尿異常のない健常対照者32名のGd-IgA1値をELISAで測定し比較した. Gd-IgA1値は, 家系内構成者群(n=34)で有意に高値であった(P<0.001). 家系内構成者をIgA腎症患者群(n=16)と家系内血縁者に分けて対照群と比較すると, 対照群, 家系内血縁者群, IgA腎症患者群の順にGd-IgA1が上昇する傾向が認められ, IgA腎症患者群は対照群に比較して有意に高値であった(P<0.001). また家系内平均Gd-IgA1値が高い家系は明らかに浸透率が高い常染色体優性遺伝様式を示した. 腎生検所見を検討し得る家族性IgA腎症患者8名において, Gd-IgA1は腎生検時の尿蛋白量と有意な正の相関関係を認めた(P=0.046). 糸球体病変から算出された組織障害度については, 統計学的に有意ではないが, Gd-IgA1が高値であるほど糸球体障害度が高度である傾向が認められた(P=0.06). 日本人の家族性IgA腎症家系ではGd-IgA1は有意に高値であり, Gd-IgA1がIgA腎症の発症・進展に関与している可能性が考えられた. 家族性IgA腎症のGd-IgA1には多様性があり, 背景にある遺伝様式や原因遺伝子がそれぞれ異なる可能性が示唆された.","subitem_description_type":"Abstract"}]},"item_7_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"60492","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Wada, Masakazu"}]},{"nameIdentifiers":[{"nameIdentifier":"60493","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Narita, Ichiei"}]}]},"item_7_publisher_7":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"新潟医学会"}]},"item_7_select_19":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_select_item":"publisher"}]},"item_7_source_id_11":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AN00182415","subitem_source_identifier_type":"NCID"}]},"item_7_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"00290440","subitem_source_identifier_type":"ISSN"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"和田, 真一"}],"nameIdentifiers":[{"nameIdentifier":"60490","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"成田, 一衛"}],"nameIdentifiers":[{"nameIdentifier":"60491","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":"2019-08-06"}],"displaytype":"detail","filename":"129(7)_373-381.pdf","filesize":[{"value":"2.3 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"129(7)_373-381.pdf","url":"https://niigata-u.repo.nii.ac.jp/record/8682/files/129(7)_373-381.pdf"},"version_id":"fef34d9f-6d78-4c12-8cea-ba51f0758117"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"家族性IgA腎症","subitem_subject_scheme":"Other"},{"subitem_subject":"糖鎖不全IgA1","subitem_subject_scheme":"Other"},{"subitem_subject":"Helix aspersa","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":"家族性IgA腎症における糖鎖不全IgA1の臨床的意義","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"家族性IgA腎症における糖鎖不全IgA1の臨床的意義"},{"subitem_title":"家族性IgA腎症における糖鎖不全IgA1の臨床的意義","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","909"],"pubdate":{"attribute_name":"公開日","attribute_value":"2016-10-05"},"publish_date":"2016-10-05","publish_status":"0","recid":"8682","relation_version_is_last":true,"title":["家族性IgA腎症における糖鎖不全IgA1の臨床的意義"],"weko_creator_id":"1","weko_shared_id":null},"updated":"2022-12-15T03:42:59.747882+00:00"}