{"created":"2021-03-01T06:09:44.391228+00:00","id":5864,"links":{},"metadata":{"_buckets":{"deposit":"ed598bd6-75d3-435d-8e6c-ebd5559e1f19"},"_deposit":{"id":"5864","owners":[],"pid":{"revision_id":0,"type":"depid","value":"5864"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00005864","sets":["453:455","471:561:562"]},"item_6_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"小児QT延長症候群 : KCNH2変異保持者の発症は遅いが重症である"}]},"item_6_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2017-03-23","bibliographicIssueDateType":"Issued"},"bibliographic_titles":[{}]}]},"item_6_date_granted_51":{"attribute_name":"学位授与年月日","attribute_value_mlt":[{"subitem_dategranted":"2017-03-23"}]},"item_6_degree_grantor_49":{"attribute_name":"学位授与機関","attribute_value_mlt":[{"subitem_degreegrantor":[{"subitem_degreegrantor_name":"新潟大学"}]}]},"item_6_degree_name_48":{"attribute_name":"学位名","attribute_value_mlt":[{"subitem_degreename":"博士（医学）"}]},"item_6_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Background:In children with long QT syndrome (LQTS), risk factors for cardiac events have been reported, but age-, gender- and genotype-related differences in prognosis remain unknown in Asian countries.Methods and Results:The study examined clinical prognosis at age between 1 and 20 years in 496 LQTS patients who were genotyped as either of LQT1–3 (male, n=206). Heterozygous mutations were observed in 3 major responsible genes:KCNQ1in271,KCNH2in 192, andSCN5Ain 33 patients. LQTS-associated events were classified into 3 categories: (1) syncope (n=133); (2) repetitive torsade de pointes (TdP, n=3); and (3) cardiopulmonary arrest (CPA, n=4). The risk of cardiac events was significantly lower in LQT1 girls than boys≤12 years (HR, 0.55), whereas LQT2 female patients ≥13 years had the higher risk of cardiac events than male patients (HR, 4.60). Patients in the repetitive TdP or CPA group included 1 LQT1 female patient, 1 LQT2 male patient, and 5 LQT2 female patients. All LQT2 patients in these groups had TdP repeatedly immediately after the antecedent event. In addition, all 5 female LQT2 patients in these groups had the event after or near puberty.Conclusions:Female LQT2 children might have repeated TdP shortly after prior events, especially after puberty","subitem_description_type":"Abstract"}]},"item_6_description_5":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"学位の種類: 博士（医学）. 報告番号: 甲第4276号. 学位記番号: 新大院博（医）甲第.754号. 学位授与年月日: 平成29年3月23日","subitem_description_type":"Other"},{"subitem_description":"Circulation Journal 2016; 80(3): 696–702","subitem_description_type":"Other"}]},"item_6_description_53":{"attribute_name":"学位記番号","attribute_value_mlt":[{"subitem_description":"新大院博（医）甲第754号","subitem_description_type":"Other"}]},"item_6_dissertation_number_52":{"attribute_name":"学位授与番号","attribute_value_mlt":[{"subitem_dissertationnumber":"13101甲第4276号"}]},"item_6_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"51005","nameIdentifierScheme":"WEKO"}],"names":[{"name":"小澤, 淳一"}]}]},"item_6_publisher_7":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"新潟大学"}]},"item_6_relation_14":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_type_id":{"subitem_relation_type_id_text":"info:doi/10.1253/circj.CJ-15-0933","subitem_relation_type_select":"DOI"}}]},"item_6_rights_15":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"Copyright © 2016 THE JAPANESE CIRCULATION SOCIETY"},{"subitem_rights":"一般社団法人日本循環器学会"}]},"item_6_select_19":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_select_item":"ETD"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Ozawa, Junichi"}],"nameIdentifiers":[{"nameIdentifier":"51004","nameIdentifierScheme":"WEKO"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-08-05"}],"displaytype":"detail","filename":"h28nmk754.pdf","filesize":[{"value":"893.7 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"本文","url":"https://niigata-u.repo.nii.ac.jp/record/5864/files/h28nmk754.pdf"},"version_id":"62078548-3eb1-4860-8a72-254111ed983e"},{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-08-05"}],"displaytype":"detail","filename":"h28nmk754_a.pdf","filesize":[{"value":"193.4 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"要旨","url":"https://niigata-u.repo.nii.ac.jp/record/5864/files/h28nmk754_a.pdf"},"version_id":"93281aa8-7325-415b-8093-e404d5390bd6"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Child","subitem_subject_scheme":"Other"},{"subitem_subject":"Genetics","subitem_subject_scheme":"Other"},{"subitem_subject":"Long QT syndrome","subitem_subject_scheme":"Other"},{"subitem_subject":"Sex hormone","subitem_subject_scheme":"Other"},{"subitem_subject":"Torsade de pointes","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"eng"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"thesis","resourceuri":"http://purl.org/coar/resource_type/c_46ec"}]},"item_title":"Pediatric cohort with long QT syndrome : KCNH2 mutation carriers present late onset but severe symptoms.","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Pediatric cohort with long QT syndrome : KCNH2 mutation carriers present late onset but severe symptoms."},{"subitem_title":"Pediatric cohort with long QT syndrome : KCNH2 mutation carriers present late onset but severe symptoms.","subitem_title_language":"en"}]},"item_type_id":"6","owner":"1","path":["455","562"],"pubdate":{"attribute_name":"公開日","attribute_value":"2018-09-11"},"publish_date":"2018-09-11","publish_status":"0","recid":"5864","relation_version_is_last":true,"title":["Pediatric cohort with long QT syndrome : KCNH2 mutation carriers present late onset but severe symptoms."],"weko_creator_id":"1","weko_shared_id":2},"updated":"2022-12-15T03:39:06.133913+00:00"}