It is not known at what age lung function impairment may arise in children with cystic fibrosis (CF). We assessed lung function shortly after birth in infants with CF diagnosed by newborn screening. We performed infant lung function measurements in a prospective cohort of infants with CF and healthy controls. We assessed lung clearance index (LCI), functional residual capacity (FRC) and tidal breathing parameters. The primary outcome was prevalence and severity of abnormal lung function (±1.64 z-scores) in CF. We enrolled 53 infants with CF (mean age 7.8 weeks) and 57 controls (mean age 5.2 weeks). Compared to controls, LCI and FRC were elevated (mean difference 0.30, 95% CI 0.02–0.60; p=0.034 and 14.5 mL, 95% CI 7.7–21.3 mL; p<0.001, respectively), while ratio of time to peak tidal expiratory flow to expiratory time was decreased in infants with CF. In 22 (41.5%) infants with CF, either LCI or FRC exceeded 1.64 z-scores; three infants had both elevated LCI and FRC. Shortly after birth, abnormal lung function is prevalent in CF infants. Ventilation inhomogeneity or hyperinflation may serve as noninvasive markers to monitor CF lung disease and specific treatment effects, and could thus be used as outcome parameters for future intervention studies in this age group. Altered lung function in infants with cystic fibrosis shortly after birth http://ow.ly/qPxq30eFOkW

中文翻译：

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出生后不久囊性纤维化婴儿的肺清除指数升高

目前尚不清楚囊性纤维化 (CF) 儿童在多大年龄可能出现肺功能障碍。我们在出生后不久评估了通过新生儿筛查诊断出的 CF 婴儿的肺功能。我们在 CF 婴儿和健康对照组的前瞻性队列中进行了婴儿肺功能测量。我们评估了肺清除指数 (LCI)、功能残气量 (FRC) 和潮式呼吸参数。主要结果是 CF 中异常肺功能的患病率和严重程度（±1.64 z 分数）。我们招募了 53 名 CF 婴儿（平均年龄 7.8 周）和 57 名对照组（平均年龄 5.2 周）。与对照组相比，LCI 和 FRC 升高（平均差异 0.30，95% CI 0.02–0.60；p=0.034 和 14.5 mL，95% CI 7.7–21.3 mL；p <0.001，分别），而 CF 婴儿的呼气峰值潮气流量与呼气时间的比值降低。在 22 名 (41.5%) CF 婴儿中，LCI 或 FRC 的 z 值均超过 1.64；三名婴儿的 LCI 和 FRC 均升高。出生后不久，CF 婴儿普遍存在肺功能异常。通气不均匀或过度充气可作为监测 CF 肺病和特定治疗效果的无创标志物，因此可用作该年龄组未来干预研究的结果参数。出生后不久囊性纤维化婴儿的肺功能改变 http://ow.ly/qPxq30eFOkW 通气不均匀或过度充气可作为监测 CF 肺病和特定治疗效果的无创标志物，因此可用作该年龄组未来干预研究的结果参数。出生后不久囊性纤维化婴儿的肺功能改变 http://ow.ly/qPxq30eFOkW 通气不均匀或过度充气可作为监测 CF 肺病和特定治疗效果的无创标志物，因此可用作该年龄组未来干预研究的结果参数。出生后不久囊性纤维化婴儿的肺功能改变 http://ow.ly/qPxq30eFOkW