Developments in multiple breath washout testing in children with cystic fibrosis

Saunders, Clare; Bayfield, Katie J; Irving, Samantha; Short, Christopher; Bush, Andrew; Davies, Jane C.

doi:10.1080/03007995.2016.1268999

Cited by 19 publications

(22 citation statements)

References 72 publications

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“…18 LCI 2.5 detects small changes in CF lungs, in particular in peripheral airways, and so is considered a sensitive measure of lung disease severity. [18][19][20] Despite research into reliability, validity and responsiveness of LCI 2.5 as a longer term OM, and support of LCI for clinical trial use by the European CF Society Clinical Trial Network Standardisation Committee, 14 it is not clear whether LCI 2.5 is useful in determining differences over a short time, for example, single-ACT sessions. 14 21 22 In particular, as LCI 2.5 is altered by airway obstruction, the movement of secretions which occurs during ACT, alongside altered ventilation of lung segments, could result either in increased or decreased LCI 2.5 ; both directions of change have been reported in previous small studies measuring ACT effect.…”

Section: Open Accessmentioning

confidence: 99%

Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial

et al. 2020

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IntroductionAirway clearance techniques (ACTs) are a gold standard of cystic fibrosis management; however, the majority of research evidence for their efficacy is of low standard; often attributed to the lack of sensitivity from outcome measures (OMs) used historically. This randomised controlled trial (RCT) investigates these standard OMs (sputum weight, forced expiratory volume in 1 s) and new OMs (electrical impedance tomography (EIT), multiple breath washout (MBW) and impulse oscillometry (IOS)) to determine the most useful measures of ACT.Methods and analysisThis is a single-centre RCT with crossover design. Participants perform MBW, IOS and spirometry, and then are randomised to either rest or supervised ACT lasting 30–60 min. MBW, IOS and spirometry are repeated immediately afterwards. EIT and sputum are collected during rest/ACT. On a separate day, the OMs are performed with the other intervention. Primary endpoint is difference in change in OMs before and after ACT/rest. Sample size was calculated with 80% power and significance of 5% for each OM (target n=64).Ethics and disseminationEthics approval was gained from the London–Chelsea Research Ethics Committee (reference 16/LO/0995, project ID 154635). Dissemination will involve scientific conference presentation and publication in a peer-reviewed journal.Trial registration numbersISRCTN11220163 and NCT02721498.

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Section: Open Accessmentioning

confidence: 99%

Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial

et al. 2020

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“…23,24 In comparison to spirometry parameters, it's a more sensitive indicator of pulmonary disease in young patients and it starts to be increased at an early age. [25][26][27] In prospective studies, it becomes abnormal before other lung function markers. 19,24 According to Kraemer et al, 19 LCI derived from MBNW was the strongest indicator of disease progression, followed by MEF 50 and FRC pleth .…”

Section: Lung Function In Patients With Different Genotypesmentioning

confidence: 99%

Lung function deterioration in school children with cystic fibrosis

Walicka‐Serzysko¹,

Postek²,

Milczewska³

et al. 2020

Pediatric Pulmonology

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Introduction: Lung disease in cystic fibrosis (CF) begins early in life but the capabilities for detecting abnormalities of pulmonary dysfunction in children remain limited. Objective: The study aimed to evaluate the early progression of lung function by the analysis of pulmonary hyperinflation, ventilation inhomogeneity (VI), trapped gas and airway obstruction with age. Methods: One hundred CF children aged 7 to 18, divided into two groups aged 7 to 12 (n = 40) and 13 to 18 (n = 60), were enrolled. Patients performed multiple-breath nitrogen washout (MBNW) tests and plethysmography for measurements of lung clearance index (LCI), functional residual capacity (FRC pleth , FRC MBNW), volume of trapped gas (V T), total resistance, and effective and specific effective airway resistance (R eff , sR eff). Results: We obtained a positive correlation of FRC pleth , FRC MBNW , and LCI with age. A linear correlation between FRC MBNW and FRC pleth (P < .0001) was observed. VI was higher in the group of older patients (9.79 in the group aged 7-12 and 11.67 in the group aged 13-18). An increased effective specific airway resistance >2 (z-score) was present in 58% of all subjects (50% and 63.3%, respectively). Pulmonary hyperinflation (FRC pleth >2 z-score) was observed in 33% of all patients: 25% and 36.6%, respectively. Trapped gas (V T > 2 z-score) was present in 18% of all children: 30% and 10%, respectively. Conclusion: A gradual decline in lung function is associated with an increase in VI, airway obstruction, pulmonary hyperinflation and development of trapped gas. In children who cannot perform either spirometry or plethysmography, MBNW can deliver a measurement of LCI connecting with VI as well as FRC MBNW to indicate indirectly the increase of hyperinflation.

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“…Multiple breath washout (MBW), which has been shown to be sensitive to early‐onset disease progression, has therefore gained considerable interest in the CF community. Lung clearance index (LCI 2.5 ), a parameter derived from the MBW test, is sensitive to early lung disease, being abnormal in infants, young children and older patients with apparently well‐preserved spirometry . MBW‐derived indices are increasingly being explored in both clinical and research settings, trying to define the clinical utility of the LCI 2.5 in the monitoring of CF lung disease.…”

Section: Introductionmentioning

confidence: 99%

“…Lung clearance index (LCI 2.5 ), a parameter derived from the MBW test, is sensitive to early lung disease, being abnormal in infants, young children and older patients with apparently well-preserved spirometry. 8 MBW-derived indices are increasingly being explored in both clinical and research settings, trying to define the clinical utility of the LCI 2.5 in the monitoring of CF lung disease. Using MBWderived indices, any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal response to exercise could alert the CF team to recognize early lung disease and potential exercise limiting factors.…”

Section: Introductionmentioning

confidence: 99%

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

Gambazza

Guarise

Carta

et al. 2020

Pediatric Pulmonology

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Background Lung clearance index (LCI2.5) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors. Methods Patients with CF in clinical stable conditions were recruited between 2015 and 2017. Available spirometry, nitrogen multiple‐breath washout test and symptoms‐limited exercise testing performed as parts of patients' annual routine evaluation were considered for this cross‐sectional study. To describe the relationship between exercise intensity and ventilation inhomogeneity, a linear regression analysis was performed using backward elimination based on Akaike information criteria. Results Seventy‐seven patients (38 females) were included. Sacin and LCI2.5 were significantly higher in patients with an overall reduced exercise tolerance. Peak work developed during exercise was associated with body mass index (b = 5.25; 95% confidence interval [CI] = 1.53‐8.98), forced expiratory volume in 1 second (FEV1; b = 3.71; 95% CI = 1.96‐5.46), Pseudomonas aeruginosa chronic infection (b = −8.84; 95% CI = −15.84 to −1.84) but not with LCI2.5. Conclusion Exercise capacity and airflow obstruction are associated in this Italian CF cohort. Considering the greater discriminatory power of LCI2.5 over FEV1 and peak work, the Godfrey protocol without gas analysis cannot provide detailed information about lung function or efficiency. However, this incremental protocol without gas exchange measures can still provide the CF team with information about exercise tolerance and disability.

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Developments in multiple breath washout testing in children with cystic fibrosis

Abstract: Standardization of LCI is part of an ongoing collaborative, multicenter process. This review describes the background to LCI, discusses technical issues and limitations and provides examples of its utility in clinical and research contexts.

Cited by 19 publications

References 72 publications

Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial

Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial

Lung function deterioration in school children with cystic fibrosis

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

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