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Updated: Sep 8, 2025

Standardized Measurement of Nasal Membrane Transepithelial Potential Difference NPD
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2対1の応答性CFTR変異体を持つ患者におけるエレクサカフター- テザカフター- イヴァカフターに対する汗の塩化と肺機能の反応: 2つの実世界の観察研究の分析

Pierre-Régis Burgel1, Jennifer Da Silva2, Emmanuelle Girodon3

  • 1Université Paris-Cité, Institut Cochin, CNRS, INSERM, Paris, France; Respiratory Medicine and Cystic Fibrosis National Reference Centre, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; ERN-LUNG cystic fibrosis network, Frankfurt, Germany.

The Lancet. Respiratory medicine
|September 6, 2025
PubMed
まとめ

胞性線維症 (CF) と2つのETI反応性CFTR変種を有する個人は,ETI治療後の汗塩化物濃度が1つのETI患者と比較して改善している. 肺機能の改善は類似しており,現在のCFTR調節剤が最大限の効果をもたらす可能性があることを示唆しています.

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