{"created":"2021-12-10T04:07:31.897402+00:00","id":2000147,"links":{},"metadata":{"_buckets":{"deposit":"725c5737-f2b4-4ac0-9e52-b274a8afbdbb"},"_deposit":{"id":"2000147","owners":[1],"pid":{"revision_id":0,"type":"depid","value":"2000147"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:02000147","sets":["453:456","471:537:538:1639104999452"]},"author_link":[],"item_1627361970403":{"attribute_name":"出版タイプ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2020-06","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"6","bibliographicPageEnd":"205","bibliographicPageStart":"199","bibliographicVolumeNumber":"134","bibliographic_titles":[{"bibliographic_title":"新潟医学会雑誌","bibliographic_titleLang":"ja"}]}]},"item_7_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"症例は50歳代，男性．35歳時に，高血圧，心房細動を指摘された．X-6年，うっ血性心不全，大動脈弁輪拡張症にて入院し，翌X-5年3月にBentall手術，MAZE手術が施行された．その際に初めて多発性嚢胞腎，多発性肝嚢胞を指摘された．Cre 1.53mg/dL, eGFR 39mL/min/1.73m^2，総腎容積2,421mL(Mayo分類1D)，総肝容積8,614mL(Gigot分類II型)．以後，当院に通院継続したが，X-2年10月にはCre 2.44mg/dL, eGFR 23mL/min/1.73m^2, TKV 3,540mL, LV 12,674mLと，腎機能，腎腫大，肝腫大はいずれも悪化した．腎嚢胞増大の抑制を目的としてトルバプタン60mgを開始したが，十分な飲水ができず腎機能が悪化し30mgに減量した．動脈塞栓術の適応について，血液透析導入後に慎重に検討する予定であった．X-1年12月，eGFR 15mL/min/1.73m^2，未満に至りトルバプタンを中止した．X年1月，胸水の増量，低栄養が進行し入院した．栄養状態が悪化し腎機能も末期腎不全状態まで悪化したため，1か月後に緊急血液透析に導入した．しかし，腹部膨満による呼吸不全からCO_2ナルコーシスに陥り，非侵襲的陽圧換気療法でも管理困難であった．透析導入2か月後に死亡した．多発性嚢胞腎，多発性肝嚢胞の嚢胞増大は腹部膨満により低栄養や臓器の圧迫による症状を引き起こす．嚢胞の増大に対する治療は，腎・肝動脈塞栓術，外科的に嚢胞開窓術，またPLDには肝切除術，肝移植術が考慮されるが合併症の割合も低くはない．本例はいずれも十分に検討することができなかったが，多発性嚢胞腎，多発性肝嚢胞の嚢胞増大には，動脈塞栓術か外科的治療の適応について症例毎に慎重に検討すべきである．","subitem_description_language":"ja","subitem_description_type":"Abstract"}]},"item_7_publisher_7":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"新潟医学会","subitem_publisher_language":"ja"}]},"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":"0029-0440","subitem_source_identifier_type":"PISSN"}]},"item_access_right":{"attribute_name":"アクセス権","attribute_value_mlt":[{"subitem_access_right":"open access","subitem_access_right_uri":"http://purl.org/coar/access_right/c_abf2"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"酒巻, 裕一","creatorNameLang":"ja"},{"creatorName":"Sakamaki, Yuichi","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"忰田, 亮平","creatorNameLang":"ja"},{"creatorName":"Kaseda, Ryohei","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"越川, 智康","creatorNameLang":"ja"},{"creatorName":"Koshikawa, Tomoyasu","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"吉岡, 友基","creatorNameLang":"ja"},{"creatorName":"Yoshioka, Yuki","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"岡部, 正明","creatorNameLang":"ja"},{"creatorName":"Okabe, Masaaki","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"成田, 一衛","creatorNameLang":"ja"},{"creatorName":"Narita, Ichiei","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"青柳, 竜治","creatorNameLang":"ja"},{"creatorName":"Aoyagi, Ryuji","creatorNameLang":"en"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_access","date":[{"dateType":"Available","dateValue":"2021-12-10"}],"displaytype":"detail","filename":"134(6)_199-205.pdf","filesize":[{"value":"3.9MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"objectType":"fulltext","url":"https://niigata-u.repo.nii.ac.jp/record/2000147/files/134(6)_199-205.pdf"},"version_id":"b0d04da4-0031-4495-897e-e3d55783b044"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"多発性嚢胞腎","subitem_subject_language":"ja","subitem_subject_scheme":"Other"},{"subitem_subject":"多発性肝嚢胞","subitem_subject_language":"ja","subitem_subject_scheme":"Other"},{"subitem_subject":"トルバプタン","subitem_subject_language":"ja","subitem_subject_scheme":"Other"},{"subitem_subject":"動脈塞栓術","subitem_subject_language":"ja","subitem_subject_scheme":"Other"},{"subitem_subject":"血液透析","subitem_subject_language":"ja","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":"多発性嚢胞腎・多発性肝嚢胞が経時的に増大し，慢性呼吸不全で死亡した1例","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"多発性嚢胞腎・多発性肝嚢胞が経時的に増大し，慢性呼吸不全で死亡した1例","subitem_title_language":"ja"},{"subitem_title":"Enlarged Polycystic Kidney Disease and Polycystic Liver Disease may lead to Chronic Respiratory Failure","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","1639104999452"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2021-12-10"},"publish_date":"2021-12-10","publish_status":"0","recid":"2000147","relation_version_is_last":true,"title":["多発性嚢胞腎・多発性肝嚢胞が経時的に増大し，慢性呼吸不全で死亡した1例"],"weko_creator_id":"1","weko_shared_id":-1},"updated":"2022-12-15T04:35:35.584687+00:00"}