The fraction of exhaled nitric oxide (FeNO) is generally lower in individuals with cystic fibrosis (CF), compared to healthy controls. Two recent studies reported that the cystic fibrosis transmembrane conductance regulator (CFTR) potentiator ivacaftor resulted in an increase in FeNO after 4 weeks’ therapy [1, 2], suggesting that changes in FeNO have the potential to serve as biomarker of restored CFTR function. Ivacaftor results in a sustained increase in FeNO in children and adults with CF. The increase in FeNO may be related to changes in airway NO metabolism by myeloperoxidase. Lumacaftor–ivacaftor therapy does not have an immediate effect on FeNO. http://bit.ly/2mzqhds

中文翻译：

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CFTR 调节剂治疗对气道一氧化氮的长期影响

与健康对照组相比，囊性纤维化 (CF) 患者呼出的一氧化氮 (FeNO) 含量通常较低。最近的两项研究报道，囊性纤维化跨膜电导调节剂 (CFTR) 增效剂 ivacaftor 导致治疗 4 周后 FeNO 增加 [1, 2]，表明 FeNO 的变化有可能作为 CFTR 功能恢复的生物标志物。Ivacaftor 导致 CF 儿童和成人的 FeNO 持续增加。FeNO的增加可能与髓过氧化物酶对气道NO代谢的改变有关。Lumacaftor-ivacaftor 治疗对 FeNO 没有立即影响。http://bit.ly/2mzqhds