Kathryn A Ramsey1,2, Tim Rosenow1,3, Lidija Turkovic1, Billy Skoric4,5, Georgia Banton1, Anne-Marie Adams4,5, Shannon J Simpson1, Conor Murray6, Sarath C Ranganathan4,5,7, Stephen M Stick1,8, Graham L Hall1. 1. 1 Telethon Kids Institute and. 2. 2 Cystic Fibrosis Research and Treatment Centre, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina. 3. 3 School of Paediatrics and Child Health, University of Western Australia, Subiaco, Australia. 4. 4 Murdoch Children's Research Institute, Parkville, Australia. 5. 5 Department of Respiratory Medicine, Royal Children's Hospital, Parkville, Australia. 6. 6 Department of Diagnostic Imaging and. 7. 7 Department of Paediatrics, University of Melbourne, Parkville, Australia. 8. 8 Department of Respiratory Medicine, Princess Margaret Hospital for Children, Subiaco, Australia; and.
Abstract
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.
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.
Authors: Sarath C Ranganathan; Graham L Hall; Peter D Sly; Stephen M Stick; Tonia A Douglas Journal: Am J Respir Crit Care Med Date: 2017-06-15 Impact factor: 21.405
Authors: Emily M DeBoer; Julia S Kimbell; Kaci Pickett; Joseph E Hatch; Kathryn Akers; John Brinton; Graham L Hall; Louise King; Fiona Ramanauskas; Tim Rosenow; Stephen M Stick; Harm A Tiddens; Thomas W Ferkol; Sarath C Ranganathan; Stephanie D Davis Journal: Respir Physiol Neurobiol Date: 2021-06-19 Impact factor: 1.931
Authors: Anne Valk; Corin Willers; Kamal Shahim; Orso Pusterla; Grzegorz Bauman; Robin Sandkühler; Oliver Bieri; Florian Wyler; Philipp Latzin Journal: Magn Reson Med Date: 2021-08-02 Impact factor: 3.737
Authors: Michelle Shaw; Umer Khan; John P Clancy; Scott H Donaldson; Scott D Sagel; Steven M Rowe; Felix Ratjen Journal: J Cyst Fibros Date: 2020-06-06 Impact factor: 5.527
Authors: Drake C Bouzek; Mahmoud H Abou Alaiwa; Ryan J Adam; Alejandro A Pezzulo; Leah R Reznikov; Daniel P Cook; Maria I Aguilar Pescozo; Patrick Ten Eyck; Chaorong Wu; Thomas J Gross; Douglas B Hornick; Eric A Hoffman; David K Meyerholz; David A Stoltz Journal: Am J Respir Crit Care Med Date: 2021-09-15 Impact factor: 30.528