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

Davies, Gwyneth; Stanojevic, Sanja; Raywood, Emma; Duncan, Julie; Stocks, Janet; Lum, Sooky; Bush, Andrew; Viviani, Laura; Wade, Angie; Calder, Alistair; Owens, Cátherine M.; Goubau, Christophe; Carr, Siobhán; Bossley, Cara; Pao, C.; Aurora, Paul

doi:10.1183/13993003.00006-2020

Cited by 21 publications

(27 citation statements)

References 15 publications

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“…Substantial therapeutic changes in the last few years with the availability of CFTR-modulators may be an additional explanation of the different LCI trajectories between these two cohorts. Our findings are similar to the reported increase in LCI of 0.18 LCI units/year from preschool to adolescence in patients with CF in the study of Davies et al [18]. While there are methodological differences to our study, the cohort assessed is clinically the most comparable currently available.…”

Section: Comparison With Literaturesupporting

confidence: 91%

“…For visit-specific covariates, we included only main effects, allowing us to assess acute changes in LCI associated with the presence of the characteristic at a given time point. Statistical analyses were performed using Stata 16.0 (StataCorp 2019) [17] and graphs created using either Stata 16.0 or Graph Pad Prism [18].…”

Section: Discussionmentioning

confidence: 99%

“…We report an increase in LCI over time between 0.24 and 0.29 LCI units/year, depending on which risk factors were adjusted for in the model. Only few studies have assessed the course of LCI over 12 months or longer, and have reported increases between 0.18 and 0.61 LCI units/year [14][15][16][17][18]. However, comparability of absolute LCI values may be limited due to the different methodologies and study designs.…”

Section: Comparison With Literaturementioning

confidence: 99%

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Longitudinal course of clinical lung clearance index in children with cystic fibrosis

et al. 2020

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RationaleWhile lung clearance index (LCI) is a sensitive marker of small airway disease in individuals with cystic fibrosis (CF), less is known about longitudinal changes in LCI during routine clinical surveillance.ObjectivesTo describe the longitudinal course of LCI in children with CF during routine clinical surveillance and assess influencing factors.MethodsChildren with CF aged 3–18 years performed LCI measurements every 3 months as part of routine clinical care between 2011 and 2018. We recorded clinical data at every visit. We used a multilevel mixed-effect model to determine changes in LCI over time and identify clinical factors that influence LCI course.Measurements and Main ResultsWe collected LCI from 1204 visits (3603 trials) in 78 participants, of which 907 visits had acceptable LCI data. The average unadjusted increase in LCI for the entire population was 0.29 LCI units·year−1 (95% CI 0.20–0.38). The increase in LCI was more pronounced in adolescence, with 0.41 units·year−1 (95% CI 0.27–0.54). Colonisation with either Pseudomonas aeruginosa or Aspergillus fumigatus, pulmonary exacerbations, CF-related diabetes, and bronchopulmonary aspergillosis were associated with a higher increase in LCI over time. Adjusting for clinical risk factors reduced the increase in LCI over time to 0.24 LCI units·year−1 (95% CI 0.16–0.33).ConclusionLCI measured during routine clinical surveillance is associated with underlying disease progression in children with CF. An increased change in LCI over time should prompt further diagnostic intervention.

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Section: Comparison With Literaturesupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Comparison With Literaturementioning

confidence: 99%

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Longitudinal course of clinical lung clearance index in children with cystic fibrosis

et al. 2020

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“…Abnormal LCI in preschool children is associated with many clinical parameters including F508del homozygous genotype, P. aeruginosa colonization and nebulised dornase alpha use. It is also predictive of spirometry in later childhood ( 56 ), as well as LCI during school age ( 57 ) and adolescence ( 58 ).…”

Section: Lung Clearance Index Lcimentioning

confidence: 99%

Toward the Establishment of New Clinical Endpoints for Cystic Fibrosis: The Role of Lung Clearance Index and Cardiopulmonary Exercise Testing

et al. 2021

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As Cystic Fibrosis (CF) treatment advances, research evidence has highlighted the value and applicability of Lung Clearance Index and Cardiopulmonary Exercise Testing as endpoints for clinical trials. In the context of these new endpoints for CF trials, we have explored the use of these two test outcomes for routine CF care. In this review we have presented the use of these methods in assessing disease severity, disease progression, and the efficacy of new interventions with considerations for future research.

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“…Early lung function tests aim to detect evidence of early lung disease. This is important for targeting appropriate treatment, and also because of its association with adverse outcomes in later childhood [23][24][25]. One of the challenges is that "early lung function", within the first years of life, is often not routinely measured in clinical practice.…”

Section: Measuring Lung Function In the Early Yearsmentioning

confidence: 99%

Does newborn screening improve early lung function in cystic fibrosis?

Davies

2022

Paediatric Respiratory Reviews

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Despite evidence showing an improvement in nutritional outcomes following diagnosis by newborn screening (NBS) for cystic fibrosis (CF), the impact on pulmonary outcomes has been less clear. In this review the approaches to measurement of early lung function and knowledge gained from NBS CF cohorts will be described. Studies which have compared outcomes in those diagnosed by NBS to those diagnosed following symptomatic presentation will be presented. Compiling the evidence base used to evaluate the impact of NBS on pulmonary outcomes has been complicated by improvements in clinical management, infection control practices, as well as public health interventions (such as tobacco smoking bans in public places) that have evolved substantially over recent decades. Forced expiratory volumes have been used as the main outcome but it is important not to draw conclusions for 'early lung function' from tests such as spirometry alone, which lack sensitivity in early lung disease. There is, at present, insufficient evidence to draw firm conclusions about the effect of NBS on early lung function. In an era of highly effective treatments targeting the underlying molecular defect responsible for CF, future opportunities for early initiation of treatment may mean that the impact of NBS on early lung function may yet to be realised.

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

Cited by 21 publications

References 15 publications

Longitudinal course of clinical lung clearance index in children with cystic fibrosis

Longitudinal course of clinical lung clearance index in children with cystic fibrosis

Toward the Establishment of New Clinical Endpoints for Cystic Fibrosis: The Role of Lung Clearance Index and Cardiopulmonary Exercise Testing

Does newborn screening improve early lung function in cystic fibrosis?

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