{"created":"2023-06-20T15:56:34.718589+00:00","id":13508,"links":{},"metadata":{"_buckets":{"deposit":"4b0b8737-4500-4360-bff0-ce9810b01939"},"_deposit":{"created_by":22,"id":"13508","owners":[22],"pid":{"revision_id":0,"type":"depid","value":"13508"},"status":"published"},"_oai":{"id":"oai:redcross.repo.nii.ac.jp:00013508","sets":["100:90:498"]},"author_link":["49599","7141","49600","49604","43347","25724","49601","49605","49602","49603","46505"],"item_5_biblio_info_7":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2017-12","bibliographicIssueDateType":"Issued"},"bibliographicPageEnd":"20","bibliographicPageStart":"14","bibliographicVolumeNumber":"38","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":"先天性凝固第V因子欠乏症は稀な常染色体劣性遺伝性疾患であり、その出血傾向は比較的に軽症とされている。我々は、胃腺腫の内視鏡治療前の血液検査においてPT(PT-INR;4.20)及びAPTTの著しい延長(78.3秒)に端を発し、第V因子の著明な活性低下(<3%)を認めるに至ったが、これまで出血傾向の既往を有さず未診断だった77歳の高齢女性症例を経験した。第V因子欠乏以外の凝固因子の異常を認めず、クロスミキシング試験で第V因子インヒビターを否定できた。調べることのできた範囲では、発端者の長女とその次女(発端者の孫)の第V因子活性は軽度に低下(それぞれ53%、38%)し、ヘテロ接合症例であり、発端者はホモ接合症例と考えられた。新鮮凍結血漿(FFP)6単位の前投与により、治療前のV因子活性は32%に上昇し、内視鏡治療は安全に施行できた。但し、本症例のように出血傾向の乏しいV因子欠乏症例の観血的処置前のFFP投与法には、未だ不明の点が多い。","subitem_description_type":"Abstract"}]},"item_5_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifierScheme":"WEKO"},{"nameIdentifierScheme":"CiNii ID","nameIdentifierURI":"http://ci.nii.ac.jp/nrid/9000004437933"}],"names":[{"name":"AKAOGI, Teruaki"}]}]},"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_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"赤荻, 照章"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"岡本, 明也"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"小森, 友紀子"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"佐々木, 奈々"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"堤, 康彦"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"河田, 英里"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"魚嶋, 伸彦"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"小林, 裕"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"釜口, 麻衣"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"鈴木, 安曇"}],"nameIdentifiers":[{},{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2018-03-16"}],"displaytype":"simple","filename":"kyo2_000093.pdf","filesize":[{"value":"2.1 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"kyo2_000093","url":"https://redcross.repo.nii.ac.jp/record/13508/files/kyo2_000093.pdf"},"version_id":"783e6df4-f51d-4b3b-96d7-fe98f2cdc7e8"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"先天性第Ⅴ因子欠乏症","subitem_subject_scheme":"Other"},{"subitem_subject":"新鮮凍結血漿（FFP)","subitem_subject_scheme":"Other"},{"subitem_subject":"内視鏡治療","subitem_subject_scheme":"Other"},{"subitem_subject":"congenital coagulation factor V deficiency","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"fresh frozen plasma (FFP)","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"endoscopic treatment","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":"明らかな出血症状を呈さず高齢に至り診断された先天性凝固第V因子欠乏症の一例","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"明らかな出血症状を呈さず高齢に至り診断された先天性凝固第V因子欠乏症の一例"},{"subitem_title":"A Case of Congenital Factor V Deficiency with no clear bleeding tendency diagnosed at an old age","subitem_title_language":"en"}]},"item_type_id":"5","owner":"22","path":["498"],"pubdate":{"attribute_name":"公開日","attribute_value":"2018-03-16"},"publish_date":"2018-03-16","publish_status":"0","recid":"13508","relation_version_is_last":true,"title":["明らかな出血症状を呈さず高齢に至り診断された先天性凝固第V因子欠乏症の一例"],"weko_creator_id":"22","weko_shared_id":-1},"updated":"2023-06-20T18:58:14.202769+00:00"}