{"created":"2024-02-13T01:12:46.294461+00:00","id":2000782,"links":{},"metadata":{"_buckets":{"deposit":"60b42a02-0394-4941-a396-c0903dcd6442"},"_deposit":{"created_by":17,"id":"2000782","owner":"17","owners":[17],"pid":{"revision_id":0,"type":"depid","value":"2000782"},"status":"published"},"_oai":{"id":"oai:yamagata.repo.nii.ac.jp:02000782","sets":["29:31:1706669033099:1706669223536"]},"author_link":[],"control_number":"2000782","item_3_alternative_title_18":{"attribute_name":"その他の言語のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Citrin deficiency: Precautions for dietary management, medium-chain fatty acid triglyceride supplementation, and intravenous infusion","subitem_alternative_title_language":"en"}]},"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 ショクジ チュウサ シボウサン ノ ホジュウ ト ユエキ ニオケル チュウイ","subitem_alternative_title_language":"ja-Kana"}]},"item_3_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2024-02-21","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"53","bibliographicPageStart":"46","bibliographicVolumeNumber":"42","bibliographic_titles":[{"bibliographic_title":"山形大学紀要. 医学 : 山形医学","bibliographic_titleLang":"ja"},{"bibliographic_title":"Bulletin of the Yamagata University. Medical science : Yamagata medical journal","bibliographic_titleLang":"ja"}]}]},"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）、成人発症シトルリン血症２型（adult-onset type 2 citrullinemia: CTLN2）を惹起する。シトリンは、主に肝で機能するアスパラギン酸グルタミン酸輸送体で、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。本症では、肝臓の解糖系が一次的に障害され、二次的にPPARαが活性低下し、グルコースと脂肪酸の利用が障害される。即ち、肝臓のエネルギー欠乏が基本的病態である。中鎖脂肪酸は肝細胞に特異的にエネルギーを供給し、NICCDやCTLN2に著効を示す。しかし、本症では、低血糖の反復、成長障害、不可逆的な脳障害や肝障害が少なからず認められることから、診断後には低炭水化物食と中鎖脂肪酸の補充が推奨される。しかるに、脳は、主にグルコースを利用し、本症では糖新生が障害されており、炭水化物は少なくとも推奨栄養所要量を摂取することが重要と考える。また、食後の血糖上昇時には、シトリン欠損症の肝細胞にとって、エネルギー源として重要な遊離脂肪酸が低下するため、中鎖脂肪酸によるエネルギーの補充を要する。中鎖脂肪酸は、肝臓の一日消費エネルギー相当量を推奨最大量とし、1/3相当量を推奨最小量とする。至適量は、食事内容や運動量などにより決められる。また、乳酸リンゲル液は、肝細胞質のNAD＋不足による乳酸の代謝障害から高乳酸血症の危険性があり、使用を回避する。診療においては、シトリン欠損症の代謝特性を理解することが重要である。","subitem_description_language":"ja","subitem_description_type":"Other"}]},"item_3_full_name_2":{"attribute_name":"著者(ヨミ)","attribute_value_mlt":[{"names":[{"name":"ハヤサカ,キヨシ","nameLang":"ja-Kana"}]}]},"item_3_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"names":[{"name":"Kiyoshi,Hayasaka","nameLang":"en"}]}]},"item_3_identifier_registration":{"attribute_name":"ID登録","attribute_value_mlt":[{"subitem_identifier_reg_text":"10.15022/0002000782","subitem_identifier_reg_type":"JaLC"}]},"item_3_publisher_32":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"山形大学","subitem_publisher_language":"ja"}]},"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_language":"ja-Kana","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_language":"en","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 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deficiency","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"adult-onset type 2 citrullinemia","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"medium-chain triglycerides","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"lactate-buffered solutions","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 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