The Lung Clearance Index detects incomplete lung function recovery with acute respiratory events in school-age children with cystic fibrosis

2020 ◽  
Author(s):  
Lucy Perrem ◽  
Sanja Stanojevic ◽  
Michelle Shaw ◽  
Renee Jensen ◽  
Julia Guido ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
School Age Children ◽  
School Age ◽  
Lung Clearance ◽  
Respiratory Events ◽  
Function Recovery ◽  
Lung Clearance Index

scholarly journals Lung clearance index during hospital admission in school-age children with cystic fibrosis

2014 ◽  
Vol 13 (6) ◽  
pp. 687-691 ◽  
Author(s):  
Liam Welsh ◽  
Christopher Nesci ◽  
Haily Tran ◽  
Marisol Tomai ◽  
Sarath Ranganathan
Keyword(s):  
Cystic Fibrosis ◽  
Hospital Admission ◽  
School Age Children ◽  
School Age ◽  
Lung Clearance ◽  
Lung Clearance Index

scholarly journals Variability of lung clearance index in clinically stable cystic fibrosis lung disease in school age children

2018 ◽  
Vol 17 (2) ◽  
pp. 236-241 ◽  
Author(s):  
Marcus Svedberg ◽  
Per M. Gustafsson ◽  
Paul D. Robinson ◽  
Monica Rosberg ◽  
Anders Lindblad
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Cystic Fibrosis Lung ◽  
School Age Children ◽  
School Age ◽  
Lung Clearance ◽  
Cystic Fibrosis Lung Disease ◽  
Lung Clearance Index

scholarly journals Elevated lung clearance index in infants with cystic fibrosis shortly after birth

2017 ◽  
Vol 50 (5) ◽  
pp. 1700580 ◽  
Author(s):  
Elisabeth Kieninger ◽  
Sophie Yammine ◽  
Insa Korten ◽  
Pinelopi Anagnostopoulou ◽  
Florian Singer ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Specific Treatment ◽  
Lung Clearance ◽  
Time To Peak ◽  
Ventilation Inhomogeneity ◽  
Lung Clearance Index ◽  
Residual Capacity ◽  
Z Scores ◽  
Noninvasive Markers

It is not known at what age lung function impairment may arise in children with cystic fibrosis (CF). We assessed lung function shortly after birth in infants with CF diagnosed by newborn screening.We performed infant lung function measurements in a prospective cohort of infants with CF and healthy controls. We assessed lung clearance index (LCI), functional residual capacity (FRC) and tidal breathing parameters. The primary outcome was prevalence and severity of abnormal lung function (±1.64 z-scores) in CF.We enrolled 53 infants with CF (mean age 7.8 weeks) and 57 controls (mean age 5.2 weeks). Compared to controls, LCI and FRC were elevated (mean difference 0.30, 95% CI 0.02–0.60; p=0.034 and 14.5 mL, 95% CI 7.7–21.3 mL; p<0.001, respectively), while ratio of time to peak tidal expiratory flow to expiratory time was decreased in infants with CF. In 22 (41.5%) infants with CF, either LCI or FRC exceeded 1.64 z-scores; three infants had both elevated LCI and FRC.Shortly after birth, abnormal lung function is prevalent in CF infants. Ventilation inhomogeneity or hyperinflation may serve as noninvasive markers to monitor CF lung disease and specific treatment effects, and could thus be used as outcome parameters for future intervention studies in this age group.

scholarly journals An 8 week open-label interventional multicenter study to evaluate the lung clearance index as endpoint for clinical trials in cystic fibrosis patients ≥8 years of age, chronically infected with Pseudomonas aeruginosa

2020 ◽  
Author(s):  
Sivagurunathan Sutharsan ◽  
Susanne Naehrig ◽  
Uwe Mellies ◽  
Christian Sieder ◽  
joerg Zeigler
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Function ◽  
Lung Disease ◽  
Small Airways ◽  
Open Label ◽  
Lung Clearance ◽  
Efficacy Trials ◽  
Inhaled Antibiotics ◽  
Lung Clearance Index

Abstract Background Forced expiratory volume in 1 second (FEV 1 ) is the only parameter currently recognized as a surrogate endpoint in cystic fibrosis (CF) trials. However, FEV 1 is relatively insensitive to changes in the small airways of patients with milder lung disease. This pilot study aimed to evaluate the lung clearance index (LCI) as a marker for use in efficacy trials with inhaled antibiotics in CF. Methods This open-label, single-arm study enrolled CF patients with Pseudomonas aeruginosa infection, who were treated with tobramycin (28-day on/off regime). FEV 1 , LCI and bacterial load in sputum (CFU) were assessed at baseline, after 1, 4 and 8 weeks of treatment. Results All patients (n=17) showed elevated LCI of >11 despite 3 patients having normal FEV 1 (>90% predicted) at baseline. Overall, LCI improved in 8 (47%) patients and FEV 1 in 9 (53%) patients. At week 4, LCI improved by 0.88, FEV 1 increased by 0.52%, and P. aeruginosa reduced by 30481.3 CFU/mL. These changes were however statistically non-significant. Six adverse events occurred in 5/17 (29.4%) patients, most of which were mild-to-moderate in severity. Conclusions Due to the low evaluable sample size, no specific trend was observed related to the changes between LCI, FEV1 and CFU. Based on the individual data from this study and from recently published literature, LCI has been shown to be a more sensitive parameter than FEV1 for lung function. LCI can hypothesized to be an appropriate endpoint for efficacy trials in CF patients if the heterogeneity in lung function is limited by enrolling younger patients or patients with more milder lung disease and thus, limiting the ventilation inhomogeneities. Trial registration : The study is registered with ClinicalTrials.gov, identifier: NCT02248922

Effect of late preterm birth on lung clearance index and respiratory physiology in school‐age children

2019 ◽  
Author(s):  
Karin Yaacoby‐Bianu ◽  
Moran T. Plonsky ◽  
Michal Gur ◽  
Ronen Bar‐Yoseph ◽  
Amir Kugelman ◽  
...  
Keyword(s):  
Preterm Birth ◽  
Respiratory Physiology ◽  
Late Preterm ◽  
School Age Children ◽  
School Age ◽  
Lung Clearance ◽  
Late Preterm Birth ◽  
Lung Clearance Index
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