Background

Progression of structural lung disease (SLD) is a major risk factor for morbidity in patients with cystic fibrosis (CF). We studied changes in SLD and correlations with spirometry and nitrogen multiple breath washout (N₂MBW) outcomes to explore associations in contemporary evolution between structural and functional abnormalities in CF lung disease.

Methods

Spirometry-controlled chest-CTs using PRAGMA-CF for scoring extent of SLD, spirometry, and N₂MBW were performed at two-year intervals in school-age children with CF.

Results

Fifty-seven children aged 6–18 years were included. No significant progression in mean PRAGMA-CF scores was observed. Half of the children showed improvement in the proportion of bronchiectasis (%Bx). Lung Clearance Index (LCI) and the second moment ratio (M2) increased significantly and baseline values correlated significantly with SLD at follow-up (p ≤ 0.0002). The correlation between the change in M2 (∆M2) and the change in total SLD was R = 0.27 (p = 0.048). We found high negative predictive values (100%) for ∆M2<10% to exclude progression in SLD. For stable or improving values of LCI and M2, the predicted probability for progression in SLD was 16% and 14%, respectively (upper 95% confidence limit: 33%). Evolution in N₂MBW and CT outcomes was discordant in half of the children.

Conclusions

We found no progression in SLD over 2 years in school-age children with CF, in contrast to both LCI and M2, which along with discordant outcomes in half of the children underlines that N₂MBW and CT assess different aspects of CF lung disease. However, stable outcomes from N₂MBW were associated with stable structural lung disease.

中文翻译：

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囊性纤维化儿童反复多次呼吸冲洗测量与计算机断层扫描评分之间的前瞻性纵向关联

背景

结构性肺病 (SLD) 的进展是囊性纤维化 (CF) 患者发病的主要危险因素。我们研究了 SLD 的变化以及与肺活量测定和氮多次呼吸冲洗 (N ₂ MBW) 结果的相关性，以探索 CF 肺病结构和功能异常之间当代演变的关联。

方法

在患有 CF 的学龄儿童中，每隔两年进行一次肺活量控制胸部 CT，使用 PRAGMA-CF 对 SLD、肺活量和 N _{2 MBW 的评分范围进行评分。}

结果

包括 57 名 6-18 岁的儿童。没有观察到平均 PRAGMA-CF 评分的显着进展。半数儿童的支气管扩张比例（%Bx）有所改善。肺清除指数 (LCI) 和二阶矩比 (M2) 显着增加，基线值与随访时的 SLD 显着相关 ( p  ≤ 0.0002)。M2 (ΔM2) 变化与总 SLD 变化之间的相关性为R  = 0.27 ( p  = 0.048)。我们发现 ΔM2<10% 的高阴性预测值 (100%) 可排除 SLD 的进展。对于 LCI 和 M2 的稳定或改善值，SLD 进展的预测概率分别为 16% 和 14%（95% 置信上限：33%）。_{N 2}中的进化一半儿童的 MBW 和 CT 结果不一致。

结论

与 LCI 和 M2 相比，我们发现 CF 学龄儿童在 2 年内 SLD 没有进展，这与一半儿童的不一致结果强调 N ₂ MBW 和 CT 评估 CF 肺病的不同方面。然而，N ₂ MBW 的稳定结果与稳定的结构性肺病相关。