{"created":"2021-03-01T06:12:13.735675+00:00","id":8223,"links":{},"metadata":{"_buckets":{"deposit":"e155c130-1a04-4d34-a184-25956e503ea7"},"_deposit":{"id":"8223","owners":[],"pid":{"revision_id":0,"type":"depid","value":"8223"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00008223","sets":["453:456","471:537:538:884"]},"item_7_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Eculizumab for Paroxysmal Nocturnal Hemoglobinuria : Its effect and refractory patient"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2017-08","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"8","bibliographicPageEnd":"463","bibliographicPageStart":"457","bibliographicVolumeNumber":"131","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":"発作性夜間ヘモグロビン尿症(paroxysmal nocturnal hemoglobinuria:PNH)はphosphatidylinositol glycan class A 遺伝子に後天的変異を来した造血幹細胞がクローン性に拡大し，補体介在性の血管内溶血を来す希少疾患である．根治的治療は造血幹細胞移植で適応は重症例に限られ，その他の症例に対しては対症療法が行われてきたが，抗補体C5モノクローナル抗体であるエクリズマブが開発され，PNH患者の予後，quality of lifeは劇的に改善した．しかしながら，本邦では少数ながらエクリズマブ不応例が認められ，その原因がC5遺伝子多型によること，この多型が日本人特有であることが見出された．この知見は生物学的製剤不応性の一因および人種差により治療反応性が異なる可能性があることを明らかにした点において重要と考えられた．","subitem_description_type":"Abstract"}]},"item_7_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"57741","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Kobayashi, Hironori"}]}]},"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":"小林, 弘典"}],"nameIdentifiers":[{"nameIdentifier":"57740","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":"131(8)_457-463.pdf","filesize":[{"value":"2.4 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"131(8)_457-463.pdf","url":"https://niigata-u.repo.nii.ac.jp/record/8223/files/131(8)_457-463.pdf"},"version_id":"ebc70b52-c9be-4f40-a63e-fff86117c0fa"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"発作性夜間ヘモグロビン尿症","subitem_subject_scheme":"Other"},{"subitem_subject":"エクリズマブ","subitem_subject_scheme":"Other"},{"subitem_subject":"補体","subitem_subject_scheme":"Other"},{"subitem_subject":"遺伝子多型","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":"4 血液内科領域 発作性夜間ヘモグロビン尿症とEculizumab : 効果と不応例について （シンポジウム 各科領域における生物学的製剤の現況と展望, 第720回新潟医学会）","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"4 血液内科領域 発作性夜間ヘモグロビン尿症とEculizumab : 効果と不応例について （シンポジウム 各科領域における生物学的製剤の現況と展望, 第720回新潟医学会）"},{"subitem_title":"4 血液内科領域 発作性夜間ヘモグロビン尿症とEculizumab : 効果と不応例について （シンポジウム 各科領域における生物学的製剤の現況と展望, 第720回新潟医学会）","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","884"],"pubdate":{"attribute_name":"公開日","attribute_value":"2018-02-28"},"publish_date":"2018-02-28","publish_status":"0","recid":"8223","relation_version_is_last":true,"title":["4 血液内科領域 発作性夜間ヘモグロビン尿症とEculizumab : 効果と不応例について （シンポジウム 各科領域における生物学的製剤の現況と展望, 第720回新潟医学会）"],"weko_creator_id":"1","weko_shared_id":null},"updated":"2022-12-15T03:42:36.462371+00:00"}