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Updated: Sep 10, 2025

Using Multi-fluorinated Bile Acids and In Vivo Magnetic Resonance Imaging to Measure Bile Acid Transport
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産生欠陥の治療: 胆酸合成の産生欠陥の例

Peter T Clayton1, Rohit Hirachan2, Elaine Murphy3

  • 1Inborn Errors of Metabolism, Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, London, UK.

Journal of inherited metabolic disease
|August 23, 2025
PubMed
まとめ

チェノドオキシコール酸 (CDCA) やホリック酸 (CA) のような胆汁代謝剤を用いた代替治療は,胆汁酸合成に影響する特定の先天性代謝異常,特に乳児における肝疾患や,脳突起性キサントマトーシス (CTX) などの疾患における神経学的症状を効果的に治療することができる.

キーワード:
胆酸合成障害チェノデオキシコール酸コリック酸肝臓疾患神経学的障害トリートメント

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