{"created":"2023-06-20T14:25:39.009961+00:00","id":829,"links":{},"metadata":{"_buckets":{"deposit":"a49e4c4d-1518-4e05-b5a0-4a442a0ae57e"},"_deposit":{"created_by":3,"id":"829","owners":[3],"pid":{"revision_id":0,"type":"depid","value":"829"},"status":"published"},"_oai":{"id":"oai:dmu.repo.nii.ac.jp:00000829","sets":["81:53"]},"author_link":["3572","3566","3569","3568","3574","3571","3575","3567","3573","3570"],"item_2_biblio_info_12":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2010-03-25","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"39","bibliographicPageStart":"35","bibliographicVolumeNumber":"37","bibliographic_titles":[{"bibliographic_title":"Dokkyo journal of medical sciences"}]}]},"item_2_description_10":{"attribute_name":"抄録(日)","attribute_value_mlt":[{"subitem_description":"Werdnig-Hoffmann病(WH)は乳児期早期に発症する進行性の筋萎縮を主体とする常染色体劣性疾患で,知的な障害を認めない.生命予後は不良であり2歳までに呼吸筋力の低下に伴う呼吸不全のため死亡する.我々はWHと遺伝子診断された男児の終末期ケアを経験した.児の疾患および終末期ケアについて,両親との医療面接により十分な説明および話し合いを行なった.その結果,両親より「体に侵襲のある気管切開手術や人工呼吸管理を含めた延命処置は希望しない」との方針が示され,我々も同意した.その後,児は6か月で重度の誤嚥性肺炎を発症し入院管理を要した.排痰の目的で気管内挿管を施行し,急性呼吸不全に対し酸素投与を施行した.気管内挿管後は適時サクションチューブから喀痰の喀出につとめた.栄養は経鼻胃管を用いた.児は入院から6病日目に家族や知人に見守られる中,母親に抱かれながら永眠した.WHの終末期ケアについてガイドラインはない.医療者は個々の症例に応じて家族と児の最大の利益を考慮した終末期ケアを励行することが肝要である.その際,両親へのインフォームド・コンセントに際し,挿管管理と人工呼吸管理を同様に説明するのではなく,本例のように気管内挿管を施行しかつ人工呼吸管理はしないという選択肢もあり,一考を要する.","subitem_description_type":"Other"}]},"item_2_description_11":{"attribute_name":"抄録(英)","attribute_value_mlt":[{"subitem_description":"Werdnig-Hoffmann disease (WH) develops progressiveamyotrophy in the early infantile stage and does not progressto mental retardation. The heredity of WH is autosomalrecessive. The life prognosis is poor, and breathing muscularstrength deteriorates by 2 years of age and the infantdies of respiratory failure. This report describes the hospicecare of the boy of WH. A medical interview was providedto the parents concerning hospice care of the child beforehand.The parents did not want the patient to undergo atracheal surgical operation, or receive artificial respirationmanagement, or life-prolonging treatment, and, after informedconsent, the medical staff agreed to the parents requests.Afterwards, the child developed severe aspirationrelatedpneumonia at six months of age and thus requiredhospitalization. The patient was treated with tracheal intubationfor acute respiratory failure and administered oxygen.The condition of a patient was temporarily stable aftertracheal incubation. He thereafter received nasogastric tubefeeding. Unfortunately, the child died in six days after indergoingtracheal intubation.There are no guidelines for the hospice care of patientswith WH. It is important that the medical staff provideshospice care that provides the maximum benefit to the familyand the child depending on each individual case. Informedconsent should be obtained for the parents, with regardto intubation and artificial respiration. The decisionsconcerning intubation and artificial respiratory managementare important issues of medical ethics.","subitem_description_type":"Other"}]},"item_2_description_8":{"attribute_name":"記事種別(日)","attribute_value_mlt":[{"subitem_description":"症例報告","subitem_description_type":"Other"}]},"item_2_source_id_1":{"attribute_name":"雑誌書誌ID","attribute_value_mlt":[{"subitem_source_identifier":"AA00629581"}]},"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":"獨協医科大学小児科学"},{"subitem_text_value":"獨協医科大学小児科学"},{"subitem_text_value":"獨協医科大学小児科学"},{"subitem_text_value":"獨協医科大学小児科学 : 埼玉医科大学小児科"},{"subitem_text_value":"獨協医科大学小児科学"}]},"item_2_text_7":{"attribute_name":"著者所属(英)","attribute_value_mlt":[{"subitem_text_language":"en","subitem_text_value":"Department of Pediatrics, Dokkyo Medical University"},{"subitem_text_language":"en","subitem_text_value":"Department of Pediatrics, Dokkyo Medical University"},{"subitem_text_language":"en","subitem_text_value":"Department of Pediatrics, Dokkyo Medical University"},{"subitem_text_language":"en","subitem_text_value":"Department of Pediatrics, Dokkyo Medical University : Department of Pediatrics, Saitama Medical University"},{"subitem_text_language":"en","subitem_text_value":"Department of Pediatrics, Dokkyo Medical University"}]},"item_2_title_3":{"attribute_name":"論文名よみ","attribute_value_mlt":[{"subitem_title":"キカン ナイ ソウカン ノ アトニ ジンコウ コキュウ カンリ オ シコウ シナカッタ ウェルドニッヒ・ホフマン ビョウ ノ ニュウジ レイ "}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"今高, 城治"},{"creatorName":"イマタカ, ジョウジ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"奥谷, 真由子"},{"creatorName":"オクヤ, マユコ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"塚田, 佳子"},{"creatorName":"ツカダ, ケイコ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"山内, 秀雄"},{"creatorName":"ヤマノウチ, ヒデオ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"有阪, 治"},{"creatorName":"アリサカ, オサム","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Imataka, George","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Okuya, Mayuko","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Tsukada, Keiko","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Yamanouchi, Hideo","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Arisaka, Osamu","creatorNameLang":"en"}],"nameIdentifiers":[{}]}]},"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","filename":"KJ00006372759.pdf","filesize":[{"value":"362.5 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"KJ00006372759.pdf","url":"https://dmu.repo.nii.ac.jp/record/829/files/KJ00006372759.pdf"},"version_id":"29311410-1adf-4743-80a7-a4da67b72b24"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"ウェルドニッヒ・ホフマン病"},{"subitem_subject":"終末期ケア"},{"subitem_subject":"インフォームド・コンセント"},{"subitem_subject":"筋萎縮性側索硬化症"},{"subitem_subject":"生命倫理"}]},"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":"気管内挿管の後に人工呼吸管理を施行しなかったウェルドニッヒ・ホフマン病の乳児例","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"気管内挿管の後に人工呼吸管理を施行しなかったウェルドニッヒ・ホフマン病の乳児例"},{"subitem_title":"A baby with Werdnig-Hoffmann Disease that did not Undergo Artificial Respiration Management after Tracheal Intubation","subitem_title_language":"en"}]},"item_type_id":"2","owner":"3","path":["53"],"pubdate":{"attribute_name":"公開日","attribute_value":"2017-05-24"},"publish_date":"2017-05-24","publish_status":"0","recid":"829","relation_version_is_last":true,"title":["気管内挿管の後に人工呼吸管理を施行しなかったウェルドニッヒ・ホフマン病の乳児例"],"weko_creator_id":"3","weko_shared_id":3},"updated":"2023-06-20T16:18:06.320385+00:00"}