{"created":"2021-03-01T06:07:53.116635+00:00","id":4099,"links":{},"metadata":{"_buckets":{"deposit":"6c6f9092-ef0c-4919-b1b2-ae3b24b35233"},"_deposit":{"id":"4099","owners":[],"pid":{"revision_id":0,"type":"depid","value":"4099"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00004099","sets":["453:454","482:483:484"]},"item_5_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2005-04","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"4","bibliographicPageEnd":"768","bibliographicPageStart":"763","bibliographicVolumeNumber":"94","bibliographic_titles":[{"bibliographic_title":"日本内科学会雑誌"},{"bibliographic_title":"日本内科学会雑誌","bibliographic_titleLang":"en"}]}]},"item_5_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"肺胞蛋白症（Pulmonary alveolar proteinosis, PAP)は肺胞および呼吸細気管支内にサーファクタント(Surfactant, SF)が貯留する希な肺疾患である。臨床的には先天性，二次性，特発性に分類される．二次性は血液疾患や感染症に続発し，特発性は抗GM-CSF自己抗体により発症する．　GM-CSF欠損マウスの研究により，GM-CSFシグナル異常，肺胞マクロファージの成熟障害，サーファクタントの代謝障害が主要な病態であることがわかった．また，特発性で出現する自己抗体は，GM-CSFを強力に中和しPAPを発症する．これらの研究より，肺におけるGM-CSFの重要な役割が明らかになった．治療として全肺洗浄が有効であるが，近年はGM-CSF療法が良好な成績をあげている．本稿では，肺内におけるGM-CSFの役割とPAPの病態，最近の治療法について概説する．","subitem_description_type":"Abstract"}]},"item_5_publisher_7":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"日本内科学会"}]},"item_5_select_19":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_select_item":"publisher"}]},"item_5_source_id_11":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AN00195836","subitem_source_identifier_type":"NCID"}]},"item_5_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"00215384","subitem_source_identifier_type":"ISSN"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"渡辺, 雅人"}],"nameIdentifiers":[{"nameIdentifier":"47463","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"中田, 光"}],"nameIdentifiers":[{"nameIdentifier":"47464","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":"2019-07-30"}],"displaytype":"detail","filename":"ZI_94(4)_763-768.pdf","filesize":[{"value":"2.1 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"ZI_94(4)_763-768.pdf","url":"https://niigata-u.repo.nii.ac.jp/record/4099/files/ZI_94(4)_763-768.pdf"},"version_id":"5763d7da-2630-4f4d-abd2-683bc070bc9a"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"肺胞蛋白症","subitem_subject_scheme":"Other"},{"subitem_subject":"GM-CSF","subitem_subject_scheme":"Other"},{"subitem_subject":"抗GM-CSF自己抗体","subitem_subject_scheme":"Other"},{"subitem_subject":"GM-CSF呼入療法","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":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"肺胞蛋白症の病態と治療（医学と治療の最前線）","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"肺胞蛋白症の病態と治療（医学と治療の最前線）"},{"subitem_title":"肺胞蛋白症の病態と治療（医学と治療の最前線）","subitem_title_language":"en"}]},"item_type_id":"5","owner":"1","path":["454","484"],"pubdate":{"attribute_name":"公開日","attribute_value":"2012-05-16"},"publish_date":"2012-05-16","publish_status":"0","recid":"4099","relation_version_is_last":true,"title":["肺胞蛋白症の病態と治療（医学と治療の最前線）"],"weko_creator_id":"1","weko_shared_id":null},"updated":"2022-12-15T03:37:07.304040+00:00"}