Abstract：
BACKGROUND: Effective detection of early lung disease in cystic fibrosis (CF) is critical to understanding early pathogenesis and evaluating early intervention strategies. We aimed to compare ability of several proposed sensitive functional tools to detect early CF lung disease as defined by CT structural disease in school aged children.
METHODS: 50 CF subjects (mean±SD 11.2 ± 3.5y, range 5-18y) with early lung disease (FEV1≥70 % predicted: 95.7 ± 11.8 %) performed spirometry, Multiple breath washout (MBW, including trapped gas assessment), oscillometry, cardiopulmonary exercise testing (CPET) and simultaneous spirometer-directed low-dose CT imaging. CT data were analysed using well-evaluated fully quantitative software for bronchiectasis and air trapping (AT).
RESULTS: CT bronchiectasis and AT occurred in 24 % and 58 % of patients, respectively. Of the functional tools, MBW detected the highest rates of abnormality: Scond 82 %, MBWTG RV 78 %, LCI 74 %, MBWTG IC 68 % and Sacin 51 %. CPET VO2peak detected slightly higher rates of abnormality (9 %) than spirometry-based FEV1 (2 %). For oscillometry AX (14 %) performed better than Rrs (2 %) whereas Xrs and R5-19 failed to detect any abnormality. LCI and Scond correlated with bronchiectasis (r = 0.55-0.64, p < 0.001) and AT (r = 0.73-0.74, p < 0.001). MBW-assessed trapped gas was detectable in 92 % of subjects and concordant with CT-assessed AT in 74 %.
CONCLUSIONS: Significant structural and functional deficits occur in early CF lung disease, as detected by CT and MBW. For MBW, additional utility, beyond that offered by LCI, was suggested for Scond and MBW-assessed gas trapping. Our study reinforces the complementary nature of these tools and the limited utility of conventional oscillometry and CPET in this setting.
METHODS: 50 CF subjects (mean±SD 11.2 ± 3.5y, range 5-18y) with early lung disease (FEV1≥70 % predicted: 95.7 ± 11.8 %) performed spirometry, Multiple breath washout (MBW, including trapped gas assessment), oscillometry, cardiopulmonary exercise testing (CPET) and simultaneous spirometer-directed low-dose CT imaging. CT data were analysed using well-evaluated fully quantitative software for bronchiectasis and air trapping (AT).
RESULTS: CT bronchiectasis and AT occurred in 24 % and 58 % of patients, respectively. Of the functional tools, MBW detected the highest rates of abnormality: Scond 82 %, MBWTG RV 78 %, LCI 74 %, MBWTG IC 68 % and Sacin 51 %. CPET VO2peak detected slightly higher rates of abnormality (9 %) than spirometry-based FEV1 (2 %). For oscillometry AX (14 %) performed better than Rrs (2 %) whereas Xrs and R5-19 failed to detect any abnormality. LCI and Scond correlated with bronchiectasis (r = 0.55-0.64, p < 0.001) and AT (r = 0.73-0.74, p < 0.001). MBW-assessed trapped gas was detectable in 92 % of subjects and concordant with CT-assessed AT in 74 %.
CONCLUSIONS: Significant structural and functional deficits occur in early CF lung disease, as detected by CT and MBW. For MBW, additional utility, beyond that offered by LCI, was suggested for Scond and MBW-assessed gas trapping. Our study reinforces the complementary nature of these tools and the limited utility of conventional oscillometry and CPET in this setting.
摘要：
背景：有效检测囊性纤维化（CF）中的早期肺部疾病对于理解早期发病机制和评估早期干预策略至关重要。我们旨在比较几种拟议的敏感功能工具在学龄儿童中检测由CT结构疾病定义的早期CF肺病的能力。
方法：50名CF受试者（平均值±SD11.2±3.5y，范围5-18y）患有早期肺部疾病（FEV1≥70％预测：95.7±11.8％）进行了肺活量测定，多次呼气冲洗(MBW,包括截留气体评估)，示波法,心肺运动测试（CPET）和同时肺活量计定向低剂量CT成像。使用经过充分评估的完全定量软件对支气管扩张和空气滞留（AT）进行CT数据分析。
结果：24％和58％的患者发生CT支气管扩张和AT，分别。在功能工具中，MBW检测到的异常率最高：Scond82%，MBWTGRV78%，LCI74%,MBWTGIC68%和Sacin51%。CPETVO2peak检测到的异常率（9％）略高于基于肺活量测定的FEV1（2％）。对于示波法，AX（14％）的表现优于Rrs（2％），而Xrs和R5-19未能检测到任何异常。LCI和Scond与支气管扩张（r=0.55-0.64，p<0.001）和AT（r=0.73-0.74，p<0.001）相关。在92％的受试者中可检测到MBW评估的截留气体，在74％的受试者中与CT评估的AT一致。
结论：早期CF肺疾病会出现明显的结构和功能缺陷，由CT和MBW检测到。对于MBW，额外的实用程序，除了LCI提供的之外，还建议用于Scond和MBW评估的气体捕集。我们的研究加强了这些工具的互补性，以及在这种情况下常规示波法和CPET的有限效用。
方法：50名CF受试者（平均值±SD11.2±3.5y，范围5-18y）患有早期肺部疾病（FEV1≥70％预测：95.7±11.8％）进行了肺活量测定，多次呼气冲洗(MBW,包括截留气体评估)，示波法,心肺运动测试（CPET）和同时肺活量计定向低剂量CT成像。使用经过充分评估的完全定量软件对支气管扩张和空气滞留（AT）进行CT数据分析。
结果：24％和58％的患者发生CT支气管扩张和AT，分别。在功能工具中，MBW检测到的异常率最高：Scond82%，MBWTGRV78%，LCI74%,MBWTGIC68%和Sacin51%。CPETVO2peak检测到的异常率（9％）略高于基于肺活量测定的FEV1（2％）。对于示波法，AX（14％）的表现优于Rrs（2％），而Xrs和R5-19未能检测到任何异常。LCI和Scond与支气管扩张（r=0.55-0.64，p<0.001）和AT（r=0.73-0.74，p<0.001）相关。在92％的受试者中可检测到MBW评估的截留气体，在74％的受试者中与CT评估的AT一致。
结论：早期CF肺疾病会出现明显的结构和功能缺陷，由CT和MBW检测到。对于MBW，额外的实用程序，除了LCI提供的之外，还建议用于Scond和MBW评估的气体捕集。我们的研究加强了这些工具的互补性，以及在这种情况下常规示波法和CPET的有限效用。
