BACKGROUND With the widespread introduction of newborn screening for cystic fibrosis (CF), there has been considerable emphasis on the need to develop objective markers of lung health that can be used during infancy. We hypothesised that in a newborn screened (NBS) UK cohort, evidence of airway inflammation and infection at one year would be associated with adverse structural and functional outcomes at the same age. METHODS Infants underwent lung function testing, chest CT scan and bronchoscopy with bronchoalveolar lavage (BAL) at 1 year of age when clinically well. Microbiology cultures were also available from routine cough swabs. RESULTS 65 infants had lung function, CT and BAL. Mean (SD) lung clearance index and forced expiratory volume in 0.5 s z-scores were 0.9(1.2) and -0.6(1.1) respectively; median Brody II CF-CT air trapping score on chest CT =0 (interquartile range 0-1, maximum possible score 27). Infants isolating any significant pathogen by 1 yr of age had higher LCI z-score (mean difference 0.9; 95%CI:0.4-1.4; p = 0.001) and a trend towards higher air trapping scores on CT (p = 0.06). BAL neutrophil elastase was detectable in 23% (10/43) infants in whom BAL supernatant was available. This did not relate to air trapping score on CT. CONCLUSIONS In this UK NBS cohort at one year of age, lung and airway damage is much milder and associations between inflammation, abnormal physiology and structural changes were at best weak, contrary to our hypothesis and previously published reports. Continued follow-up will clarify longer term implications of these very mild structural, functional and inflammatory changes.

中文翻译：

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新生儿筛查患有囊性纤维化的婴儿在一年时肺部疾病的结构、功能和炎症标志物的最小变化

背景 随着囊性纤维化 (CF) 新生儿筛查的广泛采用，人们相当重视开发可在婴儿期使用的肺部健康客观标志物的必要性。我们假设在英国新生儿筛查 (NBS) 队列中，一年时气道炎症和感染的证据与同龄的不良结构和功能结果相关。方法 婴儿在 1 岁时临床情况良好时接受肺功能检查、胸部 CT 扫描和支气管镜检查和支气管肺泡灌洗（BAL）。也可从常规咳嗽拭子获得微生物培养物。结果 65例患儿肺功能、CT、BAL均完成。0.5 秒 z 值的平均 (SD) 肺清除指数和用力呼气量分别为 0.9(1.2) 和 -0.6(1.1)；胸部 CT 上的 Brody II CF-CT 空气滞留评分中位数 =0（四分位距 0-1，最大可能评分 27）。在 1 岁时分离任何重要病原体的婴儿具有更高的 LCI z 分数（平均差异 0.9；95%CI：0.4-1.4；p = 0.001）和 CT 上更高的空气滞留分数的趋势（p = 0.06）。在 23% (10/43) 的婴儿中可检测到 BAL 中性粒细胞弹性蛋白酶，其中 BAL 上清液可用。这与 CT 上的空气滞留评分无关。结论 在这个英国 NBS 队列中，在一岁时，肺和气道损伤要轻得多，炎症、异常生理和结构变化之间的关联充其量是微弱的，这与我们的假设和先前发表的报告相反。持续的随访将阐明这些非常轻微的结构、功能和炎症变化的长期影响。