{"created":"2023-06-20T15:59:57.876908+00:00","id":18137,"links":{},"metadata":{"_buckets":{"deposit":"d2962c7d-50de-4238-aec6-0a70a1ee1924"},"_deposit":{"created_by":28,"id":"18137","owners":[28],"pid":{"revision_id":0,"type":"depid","value":"18137"},"status":"published"},"_oai":{"id":"oai:redcross.repo.nii.ac.jp:00018137","sets":["172:173:675"]},"author_link":["8028","51174","8033","68850","27812","1380","8024","51176","8030","28055","68851","8036","8021","33756","27883","8040","33754","8043","11741","27810"],"item_10001_biblio_info_7":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2022-03-31","bibliographicIssueDateType":"Issued"},"bibliographicPageEnd":"45","bibliographicPageStart":"41","bibliographicVolumeNumber":"39","bibliographic_titles":[{"bibliographic_title":"日本赤十字社和歌山医療センター医学雑誌"},{"bibliographic_title":"Medical Journal of Japanese Red Cross Wakayama Medical Center","bibliographic_titleLang":"en"}]}]},"item_10001_description_5":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Wiskott-Aldrich症候群（WAS）は，WAS遺伝子の異常によって発症する，小型の血小板減少を伴う原発性免疫不全症の代表的な疾患である．重症度によっては湿疹の合併がみられ，自己免疫性疾患を合併することでも知られる．治療抵抗性の免疫性血小板減少性紫斑病(ITP)の鑑別として重要である．症例は男児で，生後3ヶ月男児に血小板減少を認め，同時に自己免疫性の溶血性貧血を認めたため，Evans症候群と診断した．ステロイド投与で貧血は速やかに改善したが，血小板減少は大量ガンマグロブリン療法やステロイド投与に反応しなかった．生後8か月から蕁麻疹が出現し，生後10か月から湿疹や皮膚感染症を繰り返したため，遺伝子解析を行い，WASと診断することができ，臍帯血移植を施行した．低年齢の自己免疫性疾患を伴う血小板減少症では大型血小板であってもWASも考慮に入れる必要がある．","subitem_description_type":"Abstract"}]},"item_10001_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{}],"names":[{"name":"Fukao, Daisuke"}]},{"nameIdentifiers":[{}],"names":[{"name":"HAMAHATA, Keigo"}]},{"nameIdentifiers":[{}],"names":[{"name":"SAKABE, Masahiko"}]},{"nameIdentifiers":[{}],"names":[{"name":"Mizuno, Shinsuke"}]},{"nameIdentifiers":[{},{}],"names":[{"name":"Nukada, 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macrothrombicytopenia","subitem_title_language":"en"}]},"item_type_id":"10001","owner":"28","path":["675"],"pubdate":{"attribute_name":"公開日","attribute_value":"2022-05-16"},"publish_date":"2022-05-16","publish_status":"0","recid":"18137","relation_version_is_last":true,"title":["Evans症候群と大型血小板を伴ったWiskott-Aldrich症候群の一例"],"weko_creator_id":"28","weko_shared_id":-1},"updated":"2023-06-20T17:46:29.614596+00:00"}