{"created":"2023-06-20T15:50:15.170010+00:00","id":2897,"links":{},"metadata":{"_buckets":{"deposit":"09315218-7a6c-4dd5-8a58-de79a7d7a59a"},"_deposit":{"created_by":22,"id":"2897","owners":[22],"pid":{"revision_id":0,"type":"depid","value":"2897"},"status":"published"},"_oai":{"id":"oai:redcross.repo.nii.ac.jp:00002897","sets":["100:90:120"]},"author_link":["6462","6463","6431","6435","6433","6461","6460","6459"],"item_5_biblio_info_7":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2011-12","bibliographicIssueDateType":"Issued"},"bibliographicPageEnd":"106","bibliographicPageStart":"101","bibliographicVolumeNumber":"32","bibliographic_titles":[{"bibliographic_title":"京都第二赤十字病院医学雑誌"},{"bibliographic_title":"Medical journal of Kyoto Second Red Cross Hospital","bibliographic_titleLang":"en"}]}]},"item_5_description_5":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"症例は4ヵ月時に後弓反張、肝脾腫、体重増加不良で発症した2型Gaucher病の男児である。生後6ヵ月時にミルクの大量誤嚥から窒息を起こし、人工呼吸管理と人工サーファクタント投与でいったん呼吸状態が改善したため抜管したが、その後もミルク誤嚥を繰り返したため7ヵ月時に気管切開術を施行した。9ヵ月頃からけいれんと無呼吸発作を頻発して入院となり、10ヵ月時に本症の診断が確定したが、酵素補充療法開始直前に呼吸不全のため死亡した。同日の病理解剖の結果、肺では肺胞内にかなり多数のGaucher細胞を認めたのに対し、脳のGaucher細胞は散見される程度で、大脳皮質全層で大部分の神経細胞が脱落していたが、脳幹部の脱落はなく部位差がみられた。本症例の肺病理所見は誤嚥性肺炎の反復に伴う肺胞腔へのGaucher細胞の浸潤を示唆すると考えられ、2型Gaucher病では病初期より積極的な呼吸管理を行って誤嚥を予防することが重要と思われた。","subitem_description_type":"Abstract"}]},"item_5_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifierScheme":"WEKO"}],"names":[{"name":"UNO, Hiroshi"}]}]},"item_5_publisher_8":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"京都第二赤十字病院"}]},"item_5_source_id_11":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AN00357778","subitem_source_identifier_type":"NCID"}]},"item_5_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"0389-4908","subitem_source_identifier_type":"ISSN"}]},"item_5_version_type_20":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"宇野, 浩史"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"長村, 敏生"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"大前, 禎毅"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"長谷川, 雅文"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"清沢, 伸幸"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"山野, 剛"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"桂, 奏"}],"nameIdentifiers":[{},{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2012-10-19"}],"displaytype":"simple","filename":"MJKSRCH32_101.PDF","filesize":[{"value":"5.2 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"kyo2_pdf000014","url":"https://redcross.repo.nii.ac.jp/record/2897/files/MJKSRCH32_101.PDF"},"version_id":"d7286803-034e-44f3-ab7a-60729a97287f"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"2 型Gaucher 病","subitem_subject_scheme":"Other"},{"subitem_subject":"乳児","subitem_subject_scheme":"Other"},{"subitem_subject":"病理解剖","subitem_subject_scheme":"Other"},{"subitem_subject":"誤嚥","subitem_subject_scheme":"Other"},{"subitem_subject":"呼吸管理","subitem_subject_scheme":"Other"},{"subitem_subject":"Type II Gaucher disease","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"infant, autopsy","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"aspiration","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"respiratory control","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":"technical report"}]},"item_title":"剖検結果から呼吸管理の重要性を再認識させられた2型Gaucher病の1例","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"剖検結果から呼吸管理の重要性を再認識させられた2型Gaucher病の1例"},{"subitem_title":"An autopsy case of type II Gaucher disease in whom the importance of respiratory control was confirmed","subitem_title_language":"en"}]},"item_type_id":"5","owner":"22","path":["120"],"pubdate":{"attribute_name":"公開日","attribute_value":"2012-10-19"},"publish_date":"2012-10-19","publish_status":"0","recid":"2897","relation_version_is_last":true,"title":["剖検結果から呼吸管理の重要性を再認識させられた2型Gaucher病の1例"],"weko_creator_id":"22","weko_shared_id":22},"updated":"2023-06-20T21:54:01.010876+00:00"}