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Original Research: Genetic and Developmental Disorders |

Assessment of Early Bronchiectasis in Young Children With Cystic Fibrosis Is Dependent on Lung VolumeEarly Bronchiectasis Is Dependent on Lung Volume

Lauren S. Mott, MBBS, DCH; Karla G. Graniel, MD; Judy Park, MBiostat; Nicholas H. de Klerk, PhD; Peter D. Sly, MBBS, MD, DSc; Conor P. Murray, MBBS, DCH; Harm A. W. M. Tiddens, MD, PhD; Stephen M. Stick, MBBChir, PhD on behalf of AREST CF*
Author and Funding Information

From the Telethon Institute for Child Health Research and Centre for Child Health Research (Drs Mott and de Klerk, Prof Stick, and Ms Park) and School of Paediatrics and Child Health (Dr Mott and Prof Stick), The University of Western Australia, Perth, WA, Australia; Department of Pediatric Pulmonology and Allergology (Dr Graniel and Prof Tiddens), Erasmus Medical Centre-Sophia Children’s Hospital, and Department of Radiology (Dr Graniel and Prof Tiddens), Erasmus Medical Centre, Rotterdam, The Netherlands; Queensland Children’s Medical Research Institute (Dr Sly), University of Queensland, Brisbane, QLD, Australia; and the Department of Diagnostic Imaging (Dr Murray) and Department of Respiratory Medicine (Prof Stick), Princess Margaret Hospital for Children, Perth, WA, Australia.

Correspondence to: Lauren S. Mott, MBBS, DCH, Telethon Institute for Child Health Research, PO Box 855, West Perth, WA, 6842, Australia, e-mail: lmott@ichr.uwa.edu.au


Profs Tiddens and Stick contributed equally to this manuscript as senior authors.

*The members of the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) can be found at www.arestcf.org.

Part of this article has been presented in abstract form (Mott L, Gonzalez-Graniel K, Murray C, Sly P, Tiddens H, Stick S; AREST CF. Eur Respir J. 2011;38:848s and Mott L, Graniel K, Park J, et al; AREST CF. Respirology. 2012:17:56).

Funding/Support: The AREST CF research program is funded by the Cystic Fibrosis Foundation Therapeutics, Inc [Sly040A, Stick090A]; the Australian Cystic Fibrosis Research Trust; and the National Health and Medical Research Council [513730], Australia. Dr Mott was supported by a short-term research fellowship from the European Respiratory Society to travel to Rotterdam, The Netherlands, to commence this study and was supported in her PhD studies by the National Health and Medical Research Council, the University of Western Australia, the Cystic Fibrosis Association of Western Australia, and the Australian Cystic Fibrosis Research Trust. Dr Graniel was supported by a grant from The Nederlandse Cystic Fibrosis Stichting.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;144(4):1193-1198. doi:10.1378/chest.12-2589
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Objective:  The aim of this study was to determine whether assessment of early CT scan-detected bronchiectasis in young children with cystic fibrosis (CF) depends on lung volume.

Methods:  This study, approved by the hospital ethics committee, included 40 young children with CF from a newborn screened population contributing paired volume-controlled inspiratory and expiratory volumetric chest CT scans acquired under general anesthesia while clinically stable. Bronchiectasis was assessed with a semiquantitative CT scan score in inspiration and expiration, and the sensitivity of the expiratory CT scan to detect bronchiectasis was compared with the inspiratory CT scan by sensitivity and intraclass correlation coefficient analysis and Bland-Altman plots. Matched inspiratory and expiratory airway-vessel measurements were obtained in a subset of 10 children, and the relationship between lung volume and airway:vessel ratio after adjusting for age and vessel size was examined with the use of a linear regression model with generalized estimating equations. The number of visible airways in inspiration and expiration was compared in all 40 children by Wilcoxon signed rank test.

Results:  Expiratory scans had poor sensitivity (0.46) to detect bronchiectasis, underestimating disease extent (P < .001). Airway:vessel ratios were consistently higher in inspiration, independent of age and vessel size (P < .001), with significantly more airways visible in inspiration than in expiration, independent of age (median, 71 vs 28, respectively; P < .001).

Conclusions:  In young children with CF, radiologic assessment of early bronchiectasis with chest CT scan depends on lung volume; thus, expiratory scans may not be appropriate for evaluating bronchiectasis in this population. Lung volume during CT image acquisition should be standardized to evaluate airway dimensions in young children.

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