An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter

doi:10.1183/13993003.00006-2020

Observational Study

. 2020 Oct 1;56(4):2000006.

doi: 10.1183/13993003.00006-2020. Print 2020 Oct.

An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter

Gwyneth Davies^{1

2

3}, Sanja Stanojevic^{4

3}, Emma Raywood⁵, Julie A Duncan⁵, Janet Stocks⁵, Sooky Lum⁵, Andrew Bush⁶, Laura Viviani⁵, Angie Wade⁷, Alistair Calder⁸, Catherine M Owens⁸, Christophe Goubau⁵, Siobhán B Carr⁶, Cara J Bossley⁹, Caroline Pao¹⁰, Paul Aurora^{5

2}; London Cystic Fibrosis Collaboration

Affiliations

¹ Infection, Immunity and Inflammation Research and Teaching Dept, UCL Great Ormond Street Institute of Child Health (UCL GOS ICH), London, UK gwyneth.davies@ucl.ac.uk.
² Dept of Respiratory Medicine, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
³ Joint first authors.
⁴ Translational Medicine, SickKids Research Institute, Toronto, ON, Canada.
⁵ Infection, Immunity and Inflammation Research and Teaching Dept, UCL Great Ormond Street Institute of Child Health (UCL GOS ICH), London, UK.
⁶ Dept of Paediatric Respiratory Medicine, Imperial College and Royal Brompton and Harefield Hospital NHS Foundation Trust, London, UK.
⁷ Clinical Epidemiology, Nutrition and Biostatistics Section, UCL GOS ICH, London, UK.
⁸ Dept of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
⁹ Dept of Paediatric Respiratory Medicine, Kings College Hospital, London, UK.
¹⁰ Dept of Paediatric Respiratory Medicine, Royal London Hospital, London, UK.

PMID: 32444409
PMCID: PMC7527650
DOI: 10.1183/13993003.00006-2020

Free PMC article

Observational Study

An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter

Gwyneth Davies et al. Eur Respir J. 2020.

Free PMC article

. 2020 Oct 1;56(4):2000006.

doi: 10.1183/13993003.00006-2020. Print 2020 Oct.

Authors

Affiliations

¹ Infection, Immunity and Inflammation Research and Teaching Dept, UCL Great Ormond Street Institute of Child Health (UCL GOS ICH), London, UK gwyneth.davies@ucl.ac.uk.
² Dept of Respiratory Medicine, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
³ Joint first authors.
⁴ Translational Medicine, SickKids Research Institute, Toronto, ON, Canada.
⁵ Infection, Immunity and Inflammation Research and Teaching Dept, UCL Great Ormond Street Institute of Child Health (UCL GOS ICH), London, UK.
⁶ Dept of Paediatric Respiratory Medicine, Imperial College and Royal Brompton and Harefield Hospital NHS Foundation Trust, London, UK.
⁷ Clinical Epidemiology, Nutrition and Biostatistics Section, UCL GOS ICH, London, UK.
⁸ Dept of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
⁹ Dept of Paediatric Respiratory Medicine, Kings College Hospital, London, UK.
¹⁰ Dept of Paediatric Respiratory Medicine, Royal London Hospital, London, UK.

PMID: 32444409
PMCID: PMC7527650
DOI: 10.1183/13993003.00006-2020

Abstract

Lung clearance index (LCI) in the early years was associated with LCI during adolescence in children with cystic fibrosis. Pre-school LCI may help to identify children in whom treatment could be intensified. https://bit.ly/2yKyMbM

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Conflict of interest statement

Conflict of interest: G. Davies reports personal fees for lectures from Chiesi, outside the submitted work. Conflict of interest: S. Stanojevic has nothing to disclose. Conflict of interest: E. Raywood has nothing to disclose. Conflict of interest: J.A. Duncan has nothing to disclose. Conflict of interest: J. Stocks has nothing to disclose. Conflict of interest: S. Lum has nothing to disclose. Conflict of interest: A. Bush has nothing to disclose. Conflict of interest: L. Viviani has nothing to disclose. Conflict of interest: A. Wade has nothing to disclose. Conflict of interest: A. Calder has nothing to disclose. Conflict of interest: C.M. Owens has nothing to disclose. Conflict of interest: C. Goubau has nothing to disclose. Conflict of interest: S.B. Carr reports non-financial support and advisory board work from Chiesi Pharmaceuticals, lecture fees from Teva, travel support, lecture fees, advisory board and steering committee work from Vertex, advisory board work from Zambon, consultancy fees from Insmed, outside the submitted work. Conflict of interest: C.J. Bossley has nothing to disclose. Conflict of interest: C. Pao has nothing to disclose. Conflict of interest: P. Aurora reports grants from CF Trust (UK), during the conduct of the study; personal fees from Vertex Pharmaceuticals, outside the submitted work.

Figures

**FIGURE 1**
Change in lung clearance index (LCI) with age in healthy subjects (HC) and those with cystic fibrosis (CF) throughout childhood. a) Rate of change in LCI with age in health (slope 0.04 (95% CI 0.002; 0.08) and CF (slope 0.18 (95% CI 0.14; 0.21). The rate of change in CF was significantly different from the healthy group (interaction coefficient −0.05 (95% CI −0.09; −0.002). Results represent mean LCI with 95% confidence intervals for the CF group (dashed line, pale shaded area) and contemporaneous controls (solid line, darker shaded area), respectively. b) Individual changes in LCI in health (solid line) and CF (dashed line).

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References

1. Aurora P, Bush A, Gustafsson P, et al. . Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis. Am J Respir Crit Care Med 2005; 171: 249–256. doi:10.1164/rccm.200407-895OC - DOI - PubMed
1. Aurora P, Stanojevic S, Wade A, et al. . Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis. Am J Respir Crit Care Med 2011; 183: 752–758. doi:10.1164/rccm.200911-1646OC - DOI - PubMed
1. Aurora P, Gustafsson P, Bush A, et al. . Multiple breath inert gas washout as a measure of ventilation distribution in children with cystic fibrosis. Thorax 2004; 59: 1068–1073. doi:10.1136/thx.2004.022590 - DOI - PMC - PubMed
1. Ranganathan SC, Stocks J, Dezateux C, et al. . The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med 2004; 169: 928–933. doi:10.1164/rccm.200309-1344OC - DOI - PubMed
1. Ranganathan SC, Dezateux C, Bush A, et al. . Airway function in infants newly diagnosed with cystic fibrosis. Lancet 2001; 358: 1964–1965. doi:10.1016/S0140-6736(01)06970-7 - DOI - PubMed

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[1] Aurora P, Bush A, Gustafsson P, et al. . Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis. Am J Respir Crit Care Med 2005; 171: 249–256. doi:10.1164/rccm.200407-895OC - DOI - PubMed

[2] Aurora P, Bush A, Gustafsson P, et al. . Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis. Am J Respir Crit Care Med 2005; 171: 249–256. doi:10.1164/rccm.200407-895OC - DOI - PubMed

[3] Aurora P, Stanojevic S, Wade A, et al. . Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis. Am J Respir Crit Care Med 2011; 183: 752–758. doi:10.1164/rccm.200911-1646OC - DOI - PubMed

[4] Aurora P, Stanojevic S, Wade A, et al. . Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis. Am J Respir Crit Care Med 2011; 183: 752–758. doi:10.1164/rccm.200911-1646OC - DOI - PubMed

[5] Aurora P, Gustafsson P, Bush A, et al. . Multiple breath inert gas washout as a measure of ventilation distribution in children with cystic fibrosis. Thorax 2004; 59: 1068–1073. doi:10.1136/thx.2004.022590 - DOI - PMC - PubMed

[6] Aurora P, Gustafsson P, Bush A, et al. . Multiple breath inert gas washout as a measure of ventilation distribution in children with cystic fibrosis. Thorax 2004; 59: 1068–1073. doi:10.1136/thx.2004.022590 - DOI - PMC - PubMed

[7] Ranganathan SC, Stocks J, Dezateux C, et al. . The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med 2004; 169: 928–933. doi:10.1164/rccm.200309-1344OC - DOI - PubMed

[8] Ranganathan SC, Stocks J, Dezateux C, et al. . The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med 2004; 169: 928–933. doi:10.1164/rccm.200309-1344OC - DOI - PubMed

[9] Ranganathan SC, Dezateux C, Bush A, et al. . Airway function in infants newly diagnosed with cystic fibrosis. Lancet 2001; 358: 1964–1965. doi:10.1016/S0140-6736(01)06970-7 - DOI - PubMed

[10] Ranganathan SC, Dezateux C, Bush A, et al. . Airway function in infants newly diagnosed with cystic fibrosis. Lancet 2001; 358: 1964–1965. doi:10.1016/S0140-6736(01)06970-7 - DOI - PubMed

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An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter

Affiliations

An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter

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Abstract

Conflict of interest statement

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