Bayfield Katie J, Weinheimer Oliver, Middleton Anna, Boyton Christie, Fitzpatrick Rachel, Kennedy Brendan, Blaxland Anneliese, Jayasuriya Geshani, Caplain Neil, Wielpütz Mark O, Yu Lifeng, Galban Craig J, Robinson Terry E, Bartholmai Brian, Gustafsson Per, Fitzgerald Dominic, Selvadurai Hiran, Robinson Paul D
The Children's Hospital at Westmead, Westmead, New South Wales, Australia.
Department of Diagnostic and Interventional Radiology, University Hospital of Heidelberg, Heidelberg, Germany; Translational Lung Research Center Heidelberg, German Center for Lung Research DZL, Heidelberg, Germany.
J Cyst Fibros. 2024 Sep;23(5):918-925. doi: 10.1016/j.jcf.2024.05.012. Epub 2024 Jul 4.
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.
50 CF subjects (mean±SD 11.2 ± 3.5y, range 5-18y) with early lung disease (FEV≥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).
CT bronchiectasis and AT occurred in 24 % and 58 % of patients, respectively. Of the functional tools, MBW detected the highest rates of abnormality: S 82 %, MBW 78 %, LCI 74 %, MBW 68 % and S 51 %. CPET VOpeak detected slightly higher rates of abnormality (9 %) than spirometry-based FEV (2 %). For oscillometry AX (14 %) performed better than Rrs (2 %) whereas Xrs and R5-19 failed to detect any abnormality. LCI and S 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 %.
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 S 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.
有效检测囊性纤维化（CF）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。我们旨在比较几种提议的敏感功能工具检测学龄儿童中由CT结构疾病定义的早期CF肺部疾病的能力。
50例患有早期肺部疾病（FEV≥预测值的70%：95.7±11.8%）的CF受试者（平均±标准差11.2±3.5岁，范围5 - 18岁）进行了肺活量测定、多次呼吸冲洗法（MBW，包括滞留气体评估）、振荡法、心肺运动试验（CPET）以及同步肺活量计引导下的低剂量CT成像。使用经过充分评估的支气管扩张和气体滞留（AT）全定量软件分析CT数据。
分别有24%和58%的患者出现CT支气管扩张和AT。在这些功能工具中，MBW检测到的异常率最高：S为82%，MBW为78%，LCI为74%，MBW为68%，S为51%。CPET的VOpeak检测到的异常率（9%）略高于基于肺活量测定的FEV（2%）。对于振荡法，AX（14%）的表现优于Rrs（2%），而Xrs和R5 - 19未检测到任何异常。LCI和S与支气管扩张（r =
