{"_buckets": {"deposit": "16b2979d-dc60-400d-8a09-09ce72f53235"}, "_deposit": {"created_by": 3, "id": "423", "owners": [3], "pid": {"revision_id": 0, "type": "depid", "value": "423"}, "status": "published"}, "_oai": {"id": "oai:dmu.repo.nii.ac.jp:00000423", "sets": ["37"]}, "author_link": ["1698", "1700", "1709", "1703", "1704", "1705", "1710", "1706", "1707", "1699", "1697", "1701", "1702", "1708"], "item_2_biblio_info_12": {"attribute_name": "書誌情報", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2006-03-25", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "1", "bibliographicPageEnd": "60", "bibliographicPageStart": "55", "bibliographicVolumeNumber": "33", "bibliographic_titles": [{"bibliographic_title": "Dokkyo journal of medical sciences"}]}]}, "item_2_description_10": {"attribute_name": "抄録(日)", "attribute_value_mlt": [{"subitem_description": "プラダー・ウィリー症候群(PWS)患者は,しばしば緊張低下,呼吸障害,間脳機能障害,重度肥満,糖尿病など諸々の死につながりうる異常を呈する症候群である.実際,突然死や肥満に関連した死亡の報告例が本疾患で認められる.しかしながら,本症の正確な死亡原因は,報告例が少ないため不明である.成長ホルモン(GH)療法が世界的に開始されGH療法と死亡との因果関係を提案したいくつかの死亡例が報告された.本研究において,我々はGH非使用中に死亡した患者13名の死亡原因を解析した.死亡の年齢は,9ヵ月から34歳まである.それらのいずれも,GH治療を受けていない.3歳以下での多くの死亡は,ウイルス感染を契機にしていた.20歳を超える群では,肥満に関連した死亡である.2名が入浴中に死亡しており,同様な事例が以前にも報告されている.入浴中の死亡は,間脳機能障害に起因する呼吸調節障害に関連すると推測されている.我々はGH療法を用いて患者で死因を解析した.GH療法中死亡した10例は,既報告の文献を使用した,GH療法中の死亡原因は,GH非使用群と類似していた.しかしながら,GH使用による閉塞性呼吸障害の増悪が認められた.これは,突然死をきたすことが判明した.我々は,この症候群において幼児のウイルス感染中には注意深い観察が,成人においては体重のコントロールが死亡頻度を減らすために欠かせないと強調する.GH開始前においては,呼吸機能のモニタリングは必須である.", "subitem_description_type": "Other"}]}, "item_2_description_11": {"attribute_name": "抄録(英)", "attribute_value_mlt": [{"subitem_description": "Patients with Prader-Willi syndrome (PWS) often develop several potentially life-threatening conditions including hypotonia, respiratory failure, hypothalamic dysfunction, massive obesity, and diabetes mellitus. Indeed, sudden deaths or obesity-related deaths are recognized with this disorder. However, exact causes have not been revealed because of the paucity of case reports of death with this syndrome. Growth hormone (GH) treatment has been started worldwide and several cases of death have been reported, suggesting a possible pathogenetic relationship between GH treatment and death. In this study we report 13 deceased patients who have not been treated with GH and searched for causes of death. Ages at death ranged from 9 months to 34 years. None of them were treated with GH. Under the age of 3 years, causes of death in general were associated with viral infection leading to rapid demise. Over the age of 20 years, causes of death were associated with overweight, Two individuals died in bathtubs and similar events have been reported previously. The tub drowning deaths could be related to poor respiratory control caused by hypotnalamic dysfunction. In the discussion we analyze causes of death in patients with the use of GH. Ten reported cases were available in the literature. Generally causes of death with GH use were similar to those without GH. However, exacerbation of obstructive respiratory disturbance by GH use was also observed. This could have caused the unexpected death. In conclusion, we emphasize that close observation during viral infection in young children and weight control in adults are essential to reduce the frequency of death in this syndrome. Monitoring of respiratory function before and after starting GH is mandatory.", "subitem_description_type": "Other"}]}, "item_2_description_15": {"attribute_name": "表示順", "attribute_value_mlt": [{"subitem_description": "9", "subitem_description_type": "Other"}]}, "item_2_description_16": {"attribute_name": "アクセション番号", "attribute_value_mlt": [{"subitem_description": "KJ00004451272", "subitem_description_type": "Other"}]}, "item_2_description_8": {"attribute_name": "記事種別(日)", "attribute_value_mlt": [{"subitem_description": "原著", "subitem_description_type": "Other"}]}, "item_2_description_9": {"attribute_name": "記事種別(英)", "attribute_value_mlt": [{"subitem_description": "Original", "subitem_description_type": "Other"}]}, "item_2_source_id_1": {"attribute_name": "雑誌書誌ID", "attribute_value_mlt": [{"subitem_source_identifier": "AA00629581", "subitem_source_identifier_type": "NCID"}]}, "item_2_source_id_19": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "03855023", "subitem_source_identifier_type": "ISSN"}]}, "item_2_text_6": {"attribute_name": "著者所属(日)", "attribute_value_mlt": [{"subitem_text_value": "獨協医科大学越谷病院 小児科"}, {"subitem_text_value": "獨協医科大学越谷病院 小児科"}, {"subitem_text_value": "獨協医科大学越谷病院 小児科"}, {"subitem_text_value": "獨協医科大学越谷病院 小児科"}, {"subitem_text_value": "獨協医科大学越谷病院 小児科"}, {"subitem_text_value": "獨協医科大学越谷病院 小児科"}, {"subitem_text_value": "獨協医科大学越谷病院 小児科"}]}, "item_2_text_7": {"attribute_name": "著者所属(英)", "attribute_value_mlt": [{"subitem_text_language": "en", "subitem_text_value": "Dokkyo University School of Medicine, Koshigaya Hospital"}, {"subitem_text_language": "en", "subitem_text_value": "Dokkyo University School of Medicine, Koshigaya Hospital"}, {"subitem_text_language": "en", "subitem_text_value": "Dokkyo University School of Medicine, Koshigaya Hospital"}, {"subitem_text_language": "en", "subitem_text_value": "Dokkyo University School of Medicine, Koshigaya Hospital"}, {"subitem_text_language": "en", "subitem_text_value": "Dokkyo University School of Medicine, Koshigaya Hospital"}, {"subitem_text_language": "en", "subitem_text_value": "Dokkyo University School of Medicine, Koshigaya Hospital"}, {"subitem_text_language": "en", "subitem_text_value": "Dokkyo University School of Medicine, Koshigaya Hospital"}]}, "item_2_title_3": {"attribute_name": "論文名よみ", "attribute_value_mlt": [{"subitem_title": "ニホンジン プラダー・ウイリー ショウコウグン カンジャ ニオケル シボウ ゲンイン ノ ケントウ : セイチョウ ホルモン チリョウ ト シイン ノ カンレン ニ ツイテノ ブンケンテキ コウサツ "}]}, "item_creator": {"attribute_name": "著者", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "堅田, 泰樹"}, {"creatorName": "カタダ, ヤスキ", "creatorNameLang": "ja-Kana"}], "nameIdentifiers": [{"nameIdentifier": "1697", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "村上, 信行"}, {"creatorName": "ムラカミ, ノブユキ", "creatorNameLang": "ja-Kana"}], "nameIdentifiers": [{"nameIdentifier": "1698", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "小幡, 一夫"}, {"creatorName": "オバタ, カズオ", "creatorNameLang": "ja-Kana"}], "nameIdentifiers": [{"nameIdentifier": "1699", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "吉野, 篤範"}, {"creatorName": "ヨシノ, アツノリ", "creatorNameLang": "ja-Kana"}], "nameIdentifiers": [{"nameIdentifier": "1700", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "高橋, 悦郎"}, {"creatorName": "タカハシ, エツロウ", "creatorNameLang": "ja-Kana"}], "nameIdentifiers": [{"nameIdentifier": "1701", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "富田, 祐造"}, {"creatorName": "トミタ, ユウゾウ", "creatorNameLang": "ja-Kana"}], "nameIdentifiers": [{"nameIdentifier": "1702", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "作田, 亮一"}, {"creatorName": "サクタ, リョウイチ", "creatorNameLang": "ja-Kana"}], "nameIdentifiers": [{"nameIdentifier": "1703", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Katada, Yasuki", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "1704", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Murakami, Nobuyuki", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "1705", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Obata, Kazuo", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "1706", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Yoshino, Atsunori", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "1707", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Takahashi, Etsurou", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "1708", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Tomita, Yuzou", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "1709", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Sakuta, Ryouichi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "1710", "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": "2017-05-24"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "KJ00004451272.pdf", "filesize": [{"value": "538.7 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 538700.0, "url": {"label": "KJ00004451272.pdf", "url": "https://dmu.repo.nii.ac.jp/record/423/files/KJ00004451272.pdf"}, "version_id": "842b93f1-791d-4cbf-99b2-af409814caf3"}]}, "item_keyword": {"attribute_name": "キーワード", "attribute_value_mlt": [{"subitem_subject": "プラダー・ウイリー症候群", "subitem_subject_scheme": "Other"}, {"subitem_subject": "突然死", "subitem_subject_scheme": "Other"}, {"subitem_subject": "肥満", "subitem_subject_scheme": "Other"}, {"subitem_subject": "成長ホルモン", "subitem_subject_scheme": "Other"}, {"subitem_subject": "呼吸障害", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Prader-willi syndrome", "subitem_subject_language": "en", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Sudden death", "subitem_subject_language": "en", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Growth hormone", "subitem_subject_language": "en", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Obesity", "subitem_subject_language": "en", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Respiratory insufficiency", "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": "journal article", "resourceuri": "http://purl.org/coar/resource_type/c_6501"}]}, "item_title": "日本人プラダー・ウイリー症候群患者における死亡原因の検討 : 成長ホルモン治療と死因の関連についての文献的考察", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "日本人プラダー・ウイリー症候群患者における死亡原因の検討 : 成長ホルモン治療と死因の関連についての文献的考察"}, {"subitem_title": "Cause of Death in the Japanese Prader-Willi syndrome With and Without Growth Hormone Treatment", "subitem_title_language": "en"}]}, "item_type_id": "2", "owner": "3", "path": ["37"], "permalink_uri": "https://dmu.repo.nii.ac.jp/records/423", "pubdate": {"attribute_name": "公開日", "attribute_value": "2017-05-24"}, "publish_date": "2017-05-24", "publish_status": "0", "recid": "423", "relation": {}, "relation_version_is_last": true, "title": ["日本人プラダー・ウイリー症候群患者における死亡原因の検討 : 成長ホルモン治療と死因の関連についての文献的考察"], "weko_shared_id": 3}