Lung Clearance Index (LCI) is Stable in Most Primary Ciliary Dyskinesia (PCD) Patients Managed in a Specialist Centre: a Pilot Study

Irving, Samantha; Carr, S.B.; Hogg, Claire; Loebinger, Michael R.; Shoemark, Amelia; Bush, Andrew

doi:10.1007/s00408-017-0022-5

Cited by 11 publications

(5 citation statements)

References 12 publications

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“…Contrary to our finding, a pilot study by Irving et al [29], which measured the LCI twice with 5-year interval in 12 PCD patients, found no significant overall change in the LCI. This difference might be because Irving's study group was simply too small to find the rather small increase in LCI, which we demonstrated; another tracer gas was used (sulphur hexafluoride); the patient cohorts might be different with respect to age, infection status, or other disease characteristics; or management practices might be different.…”

Section: Discussioncontrasting

confidence: 99%

One-year evolution and variability in multiple-breath washout indices in children and young adults with primary ciliary dyskinesia

Kobbernagel

Green

Ring

et al. 2019

European Clinical Respiratory Journal

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Background and objective: Cross-sectional and longer-term studies have demonstrated abnormal yet stable multiple-breath inert gas washout (MBW) indices in patients with primary ciliary dyskinesia (PCD). This study aimed to assess the intermediate term evolution and the betweenoccasion variability of MBW indices in PCD over 1 year. Methods: Children and young adults with a confirmed diagnosis of PCD were included in this single-centre, prospective, observational, longitudinal study. Over 1 year, nitrogen (N 2) MBW and spirometry were performed at three occasions during ordinary scheduled outpatient visits. Trends and variability in lung clearance index (LCI), moment ratios, normalized N 2 concentration at six lung volume turnovers, and regional ventilation inhomogeneity indices of the conducting and intra-acinar airways (S cond *V T and S acin *V T) were analysed using linear mixed models. Results: Forty-two patients, aged 6-29 years (median: 15.4), performed 116 N 2 MBW test occasions and 96.6% were technically acceptable. A minimal, although significant, increase in LCI over 1 year (mean: 0.51 units, 95% CI: 0.12-0.91, p = 0.01) was found; while, all other N 2 MBW indices and FEV 1 remained unchanged. A moderate correlation was observed between LCI and FEV 1 (r = −0.47, p = 0.0001). The limits of agreement between tests 1 year apart were for LCI: −1.96 to 2.98; S cond *V T : ± 0.039; S acin *V T : −0.108 to 0.128. Conclusions: Children and young adults with PCD managed at a specialist centre showed slightly, but significant, increasing LCI and otherwise unchanged ventilation inhomogeneity indices and dynamic volumes over the intermediate term of 1 year. Estimates of the variability of N 2 MBW indices may inform sample size calculations of future randomized controlled trials.

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Section: Discussioncontrasting

confidence: 99%

One-year evolution and variability in multiple-breath washout indices in children and young adults with primary ciliary dyskinesia

Kobbernagel

Green

Ring

et al. 2019

European Clinical Respiratory Journal

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“…They found no trend in LCI or FEV 1 over an average of 1 year's observation. Similar results have been reported in a small study ( n = 12) by Irving et al who compared baseline LCI values to values obtained at a five‐year follow‐up and found an overall nonsignificant change in LCI (Irving et al, 2017). To the contrary, Kobbernagel et al found a minimal, but significant worsening of LCI during a one‐year observation period, while FEV 1 remained unchanged (Kobbernagel et al, 2019).…”

Section: Pulmonary Function—evolution From Infancy To Older Age Groupssupporting

confidence: 87%

Chronic airway disease in primary ciliary dyskinesia—spiced with geno–phenotype associations

Nielsen

Holgersen

Crowley

et al. 2022

American J of Med Genetics Pt C

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Primary ciliary dyskinesia (PCD) can be defined as a multiorgan ciliopathy with a dominant element of chronic airway disease affecting the nose, sinuses, middle ear, and in particular, the lower airways. Although most patients with PCD are diagnosed during preschool years, it is obvious that the chronic lung disease starts its course already from birth. The many faces of the clinical picture change, as does lung function, structural lung damage, the burden of infection, and of treatment throughout life. A markedly severe neutrophil inflammation in the respiratory tract seems pervasive and is only to a minimal extent ameliorated by a treatment strategy, which is predominantly aimed at bacterial infections. An ever-increasing understanding of the different aspects, their interrelationships, and possible different age courses conditioned by the underlying genotype is the focus of much attention. The future is likely to offer personalized medicine in the form of mRNA therapy, but to that end, it is of utmost importance that all patients with PCD be carefully characterized and given a genetic diagnosis. In this narrative review, we have concentrated on lower airways and summarized the current understanding of the chronic airway disease in this motile ciliopathy. In addition, we highlight the challenges, gaps, and opportunities in PCD lung disease research.

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“…FEV 1 was compared to MBW-derived LCI in eight studies, also with contradictory results. Two studies reported no association [ 41 , 44 ], while the other six [ 12 , 35 , 42 , 43 , 46 , 49 ] found correlations between some parameters. Both B oon et al [ 12 ] and K obbernagel et al [ 46 ] found a significant negative correlation between LCI, FEV 1 and FEV 1 /FVC ratio z-scores, while I rving et al [ 43 ] only found a correlation between LCI and forced expiratory flow at 25–75% of FVC (FEF 25–75 ) z-scores.…”

Section: Resultsmentioning

confidence: 99%

Lower airway clinical outcome measures for use in primary ciliary dyskinesia research: a scoping review

et al. 2021

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ObjectivesDisease-specific, well-defined, and validated clinical outcome measures are essential in designing research studies. Poorly defined outcome measures hamper pooling of data and comparisons between studies. We aimed to identify and describe pulmonary outcome measures that could be used for follow-up of patients with primary ciliary dyskinesia (PCD).MethodsWe conducted a scoping review by systematically searching Medline, Embase and Cochrane Systematic Review online databases for studies published from 1996 to 2020 that included ≥10 PCD adult and/or paediatric patients.ResultsWe included 102 studies (7289 patients). Eighty-three studies reported on spirometry, 11 on body plethysmography, 15 on multiple breath washout, 36 on high-resolution computed tomography (HRCT), 57 on microbiology, and 17 on health-related quality of life. Measurement and reporting of outcomes varied considerably between studies (e.g. different scoring systems for chest HRCT scans). Additionally, definitions of outcome measures varied (e.g. definition of chronic colonisation by respiratory pathogen), impeding direct comparisons of results.ConclusionsThis review highlights the need for standardisation of measurements and reporting of outcome measures to enable comparisons between studies. Defining a core set of clinical outcome measures is necessary to ensure reproducibility of results and for use in future trials and prospective cohorts.

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Lung Clearance Index (LCI) is Stable in Most Primary Ciliary Dyskinesia (PCD) Patients Managed in a Specialist Centre: a Pilot Study

Cited by 11 publications

References 12 publications

One-year evolution and variability in multiple-breath washout indices in children and young adults with primary ciliary dyskinesia

One-year evolution and variability in multiple-breath washout indices in children and young adults with primary ciliary dyskinesia

Chronic airway disease in primary ciliary dyskinesia—spiced with geno–phenotype associations

Lower airway clinical outcome measures for use in primary ciliary dyskinesia research: a scoping review

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