Lung clearance index and structural lung disease on computed tomography in early cystic fibrosis
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Rationale: The lung clearance index is a measure of ventilation distribution derived from the multiple-breath washout technique. It has been suggested as a surrogate for chest computed tomography to detect structural lung abnormalities in individuals with cystic fibrosis (CF); however, the associations between lung clearance index and early structural lung disease are unclear. Objectives: We assessed the ability of the lung clearance index to reflect structural lung disease on the basis of chest computed tomography across the entire pediatric age range. Methods: Lung clearance index was assessed in 42 infants (ages 0–2 yr), 39 preschool children (ages 3–6 yr), and 38 school-age children (7–16 yr) with CF before chest computed tomography and in 72 healthy control subjects. Scans were evaluated for CF-related structural lung disease using the Perth-Rotterdam Annotated Grid Morphometric Analysis for Cystic Fibrosis quantitative outcome measure. Measurements and Main Results: In infants with CF, lung clearance index is insensitive to structural disease (κ = −0.03 [95% confidence interval, −0.05 to 0.16]). In preschool children with CF, lung clearance index correlates with total disease extent. In school-age children, lung clearance index correlates with extent of total disease, bronchiectasis, and air trapping. In preschool and school-age children, lung clearance index has a good positive predictive value (83–86%) but a poor negative predictive value (50–55%) to detect the presence of bronchiectasis. Conclusions: These data suggest that lung clearance index may be a useful surveillance tool to monitor structural lung disease in preschool and school-age children with CF. However, lung clearance index cannot replace chest computed tomography to screen for bronchiectasis in this population.
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The clinical utility of lung clearance index in early cystic fibrosis lung disease is not impacted by the number of multiple-breath washout trialsFoong, Rachel; Harper, A.; Skoric, B.; King, L.; Turkovic, L.; Davis, M.; Clem, C.; Rosenow, T.; Davis, S.; Ranganathan, S.; Hall, Graham; Ramsey, K. (2018)© ERS 2018. The lung clearance index (LCI) from the multiple-breath washout (MBW) test is a promising surveillance tool for pre-school children with cystic fibrosis (CF). Current guidelines for MBW testing recommend that ...
Air trapping on chest CT is associated with worse ventilation distribution in infants with cystic fibrosis diagnosed following newborn screeningHall, Graham; Logie, K.; Parsons, F.; Schulzke, S.; Nolan, G.; Murray, C.; Ranganathan, S.; Robinson, P.; Sly, P.; Stick, S. (2011)Background: In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution. The primary objective of this analysis was to determine ...
Rosenow, T.; Ramsey, K.; Turkovic, L.; Murray, C.; Mok, L.; Hall, Graham; Stick, S. (2017)Introduction: Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as “air trapping”. It is presumed to be localized hyperinflation due to small ...