{"created":"2023-06-23T12:22:04.598893+00:00","id":13872,"links":{},"metadata":{"_buckets":{"deposit":"51935b4c-039f-43f1-89ce-67f10432b40c"},"_deposit":{"created_by":21,"id":"13872","owners":[21],"pid":{"revision_id":0,"type":"depid","value":"13872"},"status":"published"},"_oai":{"id":"oai:asahi-u.repo.nii.ac.jp:00013872","sets":["46:348:490"]},"author_link":["8863","1767","4097","4109","14084","14082","4090","8865","3998","8871","496"],"item_10002_biblio_info_77":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2022-02","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"3","bibliographicPageEnd":"158","bibliographicPageStart":"153","bibliographicVolumeNumber":"48","bibliographic_titles":[{"bibliographic_title":"岐阜歯科学会雑誌"},{"bibliographic_title":"The Journal of Gifu Dental Society","bibliographic_titleLang":"en"}]}]},"item_10002_description_80":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"先天性第Ⅶ因子欠乏症は50万人に1人の頻度で発症する常染色体劣性遺伝形式を呈する稀な先天性凝固障害症の一つである。病態は外因系凝固異常を認め、内因系凝固異常は正常である。検査所見はプロトロンビン時間（PT）の延長と第Ⅶ因子活性化減少を認め、活性化部分トロンボプラスチン時間は正常である。臨床所見として血友病の様な重篤な出血傾向を示す場合は少なく、軽度もしくは無症候性の場合もあるが観血的処置の際には比較的出血は多いとも言われているため事前に十分な準備が必要である。また出血傾向を認める一方、血栓症を認める場合もある。今回われわれは先天性第Ⅶ因子欠乏症患者に発生した血管内乳頭状内皮過形成を経験した。患者は33歳男性。舌にしこりを認め当科紹介受診。臨床診断は舌血管腫。術前検査でPT延長を認め、精査し第Ⅶ因子活性化28％と減少しており先天性第Ⅶ因子欠乏症と診断された。術後、トランサミンを1g/日2日間投与した。遺伝子組み換え活性型第Ⅶ因子製剤は使用しなかった。術中、術後異常出血は認めなかった。","subitem_description_type":"Abstract"}]},"item_10002_description_81":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"Congenital factor VII deficiency is one of the rare congenital coagulation inherited in an autosomal recessive manner, with an incidence of about 1 in every 500,000. The disease is characterized by extrinsic coagulation abnormalities, while intrinsic coagulation are normal. Therefore, laboratory findings show prolonged prothrombin time and decrease activation of coagulation factor VII, while partial thromboplastin time is normal. As for clinical findings, few cases show severe bleeding tendency like hemophilia, and some cases seem to be mild or asymptomatic. However, the bleeding is relatively heavy during invasive treatment, and it is said that adequate preparation is necessary beforehand. In addition, while bleeding may occur in congenital factor VII deficiency, thrombosis may also occur. we present a case of intravascular papillary endothelial hyperplasia in a patient with congenital factor VII deficiency. The patient was a 33 years old man who referred to our hospital because of having a lump on his tongue. Congenital factor VII deficiency diagnosed preoperative investigation for surgery under general anesthesia. The serum level of factor VII was 28%. No recombinant factor VII was prepared, and tranexamic acid was administrated at a dose of 1 g/day for 2 days after surgery. No abnormal intraoperative bleeding was observed.","subitem_description_type":"Other"}]},"item_10002_full_name_75":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"8871","nameIdentifierScheme":"WEKO"}],"names":[{"name":"NAGANAWA, KOSUKE"}]},{"nameIdentifiers":[{"nameIdentifier":"14084","nameIdentifierScheme":"WEKO"}],"names":[{"name":"UKAI, AKIRA"}]},{"affiliations":[{"affiliationNames":[{"affiliationName":"朝日大学","lang":"ja"}],"nameIdentifiers":[]}],"familyNames":[{"familyName":"松原","familyNameLang":"ja"},{"familyName":"MATSUBARA","familyNameLang":"en"}],"givenNames":[{"givenName":"誠","givenNameLang":"ja"},{"givenName":"MAKOTO","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"1767","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"9000413854863","nameIdentifierScheme":"CiNii ID","nameIdentifierURI":"http://ci.nii.ac.jp/nrid/9000413854863"}],"names":[{"name":"松原, 誠","nameLang":"ja"},{"name":"MATSUBARA, 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of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University"},{"subitem_textarea_value":"朝日大学歯学部口腔病態医療学講座口腔外科学分野\nDepartment of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University"},{"subitem_textarea_value":"社会医療法人蘇西厚生会松波総合記念病院歯科口腔外科\nDepartment of Oral and Maxillofacial Surgery, Matsunami General Hospital"},{"subitem_textarea_value":"朝日大学歯学部口腔病態医療学講座口腔外科学分野\nDepartment of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University"},{"subitem_textarea_value":"朝日大学歯学部口腔病態医療学講座口腔外科学分野\nDepartment of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University"},{"subitem_textarea_value":"朝日大学歯学部口腔病態医療学講座口腔外科学分野\nDepartment of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University"},{"subitem_textarea_value":"朝日大学歯学部口腔病態医療学講座口腔外科学分野\nDepartment of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University"},{"subitem_textarea_value":"朝日大学歯学部口腔病態医療学講座口腔外科学分野\nDepartment of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University"},{"subitem_textarea_value":"朝日大学歯学部口腔病態医療学講座口腔外科学分野\nDepartment of Oral and Maxillofacial Surgery, Division of Oral Pathogenesis and Disease Control, Asahi University"}]},"item_10002_version_type_95":{"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":[{"creatorAffiliations":[{"affiliationNameIdentifiers":[],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"長縄, 鋼亮","creatorNameLang":"ja"},{"creatorName":"NAGANAWA, 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aberration","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"Thrombosis","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":"departmental bulletin paper","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"先天性第Ⅶ因子欠乏症患者の血管内乳頭状内皮過形成切除の1例","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"先天性第Ⅶ因子欠乏症患者の血管内乳頭状内皮過形成切除の1例"},{"subitem_title":"A Case of Intravascular papillary endothelial hyperplasia Resection in a Patient with Congenital Factor VII Deficiency","subitem_title_language":"en"}]},"item_type_id":"10002","owner":"21","path":["490"],"pubdate":{"attribute_name":"公開日","attribute_value":"2022-05-07"},"publish_date":"2022-05-07","publish_status":"0","recid":"13872","relation_version_is_last":true,"title":["先天性第Ⅶ因子欠乏症患者の血管内乳頭状内皮過形成切除の1例"],"weko_creator_id":"21","weko_shared_id":-1},"updated":"2024-01-16T04:55:19.733695+00:00"}