{"_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": ["455", "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", "download_preview_message": "", "file_order": 0, "filename": "h28nmk754.pdf", "filesize": [{"value": "893.7 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 893700.0, "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", "download_preview_message": "", "file_order": 1, "filename": "h28nmk754_a.pdf", "filesize": [{"value": "193.4 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 193400.0, "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"], "permalink_uri": "http://hdl.handle.net/10191/47626", "pubdate": {"attribute_name": "公開日", "attribute_value": "2018-09-11"}, "publish_date": "2018-09-11", "publish_status": "0", "recid": "5864", "relation": {}, "relation_version_is_last": true, "title": ["Pediatric cohort with long QT syndrome : KCNH2 mutation carriers present late onset but severe symptoms."], "weko_shared_id": 2}