{"created":"2023-05-25T05:26:40.973649+00:00","id":5549,"links":{},"metadata":{"_buckets":{"deposit":"f448beaa-d9a6-486a-9ed3-f5634efb9a07"},"_deposit":{"created_by":17,"id":"5549","owners":[17],"pid":{"revision_id":0,"type":"depid","value":"5549"},"status":"published"},"_oai":{"id":"oai:yamagata.repo.nii.ac.jp:00005549","sets":["29:31:355:442"]},"author_link":["16632","16633","16634"],"control_number":"5549","item_3_alternative_title_19":{"attribute_name":"タイトル（ヨミ）","attribute_value_mlt":[{"subitem_alternative_title":"シトリン ケッソンショウ：セイジン ハッショウ シトルリン ケッショウ ２ガタ ノ ハッショウ ヨウボウ オヨビ セイカツ ノ シツ ノ カイゼン ニ ツイテ"}]},"item_3_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2022-08-04","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"2","bibliographicPageEnd":"75","bibliographicPageStart":"67","bibliographicVolumeNumber":"40","bibliographic_titles":[{"bibliographic_title":"山形大学紀要. 医学 : 山形医学"},{"bibliographic_title":"ulletin of the Yamagata University. Medical science : Yamagata medical journal","bibliographic_titleLang":"en"}]}]},"item_3_description_42":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_description":"論文(Article)","subitem_description_type":"Other"}]},"item_3_description_5":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"シトリンは、主に肝臓に発現するアスパラギン酸グルタミン酸輸送体であり、肝の解糖系に不可欠なリンゴ酸－アスパラギン酸シャトルを構成する。シトリン欠損症は、新生児肝内胆汁うっ滞症（neonatal intrahepatic cholestasis caused by citrin deficiency: NICCD）、発育不全と脂質異常症 （Failure to thrive and dyslipidemia caused by citrin deficiency: FTTDCD）および成人発症シトルリン血症２型（adult-onset citrullinemia type 2: CTLN2）を惹起する。肝細胞はエネルギー源として、食後にはグルコース、食間には脂肪酸を利用するが、シトリン欠損症では解糖系の障害およびperoxisome proliferator-activated receptor α （PPARα）の活性低下による脂肪酸酸化の障害から肝細胞がエネルギー欠乏となる。肝臓は糖、脂質、アミノ酸のホメオスターシス維持に重要な役割を果たしており、発症の予防には肝細胞へのエネルギー供給が一義的に求められる。低炭水化物食はシトリン欠損症の肝細胞へエネルギーを提供する食事ではあるが、小児では体重当たり糖の必要量が多く、シトリン欠損症では糖新生も障害されることから、低血糖のリスクがある。小児では、炭水化物の必要量を充足した食事が推奨される。シトリン欠損症は、小児では低血糖症、成人では高アンモニア血症により脳に不可逆的な障害をもたらす重篤な疾患である。シトリン欠損症の病態を考慮し、CTLN2の発症予防とともに生活の質を改善する生活、特に食生活と生活上の注意事項について解説する。","subitem_description_type":"Other"}]},"item_3_full_name_2":{"attribute_name":"著者(ヨミ)","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"16633","nameIdentifierScheme":"WEKO"}],"names":[{"name":"ハヤサカ, キヨシ"}]}]},"item_3_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"16634","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Hayasaka, Kiyoshi"}]}]},"item_3_identifier_registration":{"attribute_name":"ID登録","attribute_value_mlt":[{"subitem_identifier_reg_text":"10.15022/00005464","subitem_identifier_reg_type":"JaLC"}]},"item_3_publisher_32":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"山形大学"}]},"item_3_source_id_7":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"0288-030X","subitem_source_identifier_type":"ISSN"}]},"item_3_source_id_9":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AN00067279","subitem_source_identifier_type":"NCID"}]},"item_3_text_33":{"attribute_name":"出版者（ヨミ）","attribute_value_mlt":[{"subitem_text_value":"ヤマガタ ダイガク"}]},"item_3_text_34":{"attribute_name":"別言語の出版者","attribute_value_mlt":[{"subitem_text_value":"Yamagata University"}]},"item_3_text_43":{"attribute_name":"資源タイプ・ローカル","attribute_value_mlt":[{"subitem_text_value":"紀要論文"}]},"item_3_text_44":{"attribute_name":"資源タイプ・NII","attribute_value_mlt":[{"subitem_text_value":"Departmental Bulletin Paper"}]},"item_3_text_45":{"attribute_name":"資源タイプ・DCMI","attribute_value_mlt":[{"subitem_text_value":"text"}]},"item_3_text_46":{"attribute_name":"資源タイプ・ローカル表示コード","attribute_value_mlt":[{"subitem_text_value":"2"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"早坂, 清"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_access","date":[{"dateType":"Available","dateValue":"2024-01-23"}],"filename":"kiyoum-40-2-067to075.pdf","filesize":[{"value":"1.2 MB"}],"format":"application/pdf","mimetype":"application/pdf","url":{"url":"https://yamagata.repo.nii.ac.jp/record/5549/files/kiyoum-40-2-067to075.pdf"},"version_id":"158ebf4c-ce98-4b03-b0a2-15b482734ab1"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"シトリン欠損症","subitem_subject_scheme":"Other"},{"subitem_subject":"リンゴ酸－アスパラギン酸シャトル","subitem_subject_scheme":"Other"},{"subitem_subject":"新生児肝内胆汁うっ滞症","subitem_subject_scheme":"Other"},{"subitem_subject":"成人発症シトルリン血症２型","subitem_subject_scheme":"Other"},{"subitem_subject":"中鎖脂肪酸","subitem_subject_scheme":"Other"},{"subitem_subject":"citrin deficiency","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"malate-aspartate shuttle","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"neonatal intrahepatic cholestasis caused by citrin deficiency","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"adult-onset type Ⅱ citrullinemia","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"medium chain triglycerides","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":"シトリン欠損症：成人発症シトルリン血症２型の 発症予防および生活の質の改善について","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"シトリン欠損症：成人発症シトルリン血症２型の 発症予防および生活の質の改善について","subitem_title_language":"ja"}]},"item_type_id":"3","owner":"17","path":["442"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2022-08-04"},"publish_date":"2022-08-04","publish_status":"0","recid":"5549","relation_version_is_last":true,"title":["シトリン欠損症：成人発症シトルリン血症２型の 発症予防および生活の質の改善について"],"weko_creator_id":"17","weko_shared_id":-1},"updated":"2024-01-23T04:32:59.419802+00:00"}