Background

Lung disease can develop within the first year of life in infants with cystic fibrosis (CF). However, the frequency and severity of respiratory symptoms in infancy are not known.

Methods

We assessed respiratory symptoms in 50 infants with CF and 50 healthy matched controls from two prospective birth cohort studies. Respiratory symptoms and respiratory rate were documented by standardized weekly interviews throughout the first year. Infants performed multiple breath washout in the first weeks of life.

Results

We analyzed 4552 data points (2217 in CF). Respiratory symptoms (either mild or severe) were not more frequent in infants with CF (OR:1.1;95% CI:[0.76, 1.59]; p=0.6). Higher lung clearance index and higher respiratory rate in infants with CF were not associated with respiratory symptoms.

Conclusions

We found no difference in respiratory symptoms between healthy and CF infants. These data indicate that early CF lung disease may not be captured by clinical presentation alone.

中文翻译：

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呼吸系统症状不反映早期 CF 肺病的功能障碍

背景

肺病可在患有囊性纤维化 (CF) 的婴儿出生后的第一年内发展。然而，婴儿期呼吸道症状的频率和严重程度尚不清楚。

方法

我们评估了来自两项前瞻性出生队列研究的 50 名 CF 婴儿和 50 名健康匹配对照的呼吸道症状。在第一年通过标准化的每周访谈记录呼吸症状和呼吸频率。婴儿在生命的最初几周内进行了多次呼吸冲洗。

结果

我们分析了 4552 个数据点（CF 中的 2217 个）。CF 婴儿的呼吸系统症状（轻度或重度）并不常见（OR：1.1；95% CI：[0.76, 1.59]；p=0.6）。CF 婴儿较高的肺清除指数和较高的呼吸频率与呼吸道症状无关。

结论

我们发现健康婴儿和 CF 婴儿的呼吸道症状没有差异。这些数据表明，早期 CF 肺病可能无法仅通过临床表现来捕捉。