{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T00:42:04Z","timestamp":1793148124034,"version":"4.5.0"},"reference-count":55,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2023,7,17]],"date-time":"2023-07-17T00:00:00Z","timestamp":1689552000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Properly evaluating respiratory system dysfunction is essential in children with cystic fibrosis (CF). This prospective study aimed to assess the course of early lung disease based on multiple breath nitrogen washout (MBNW), impulse oscillometry (IOS), and conventional techniques, such as spirometry and body plethysmography. Methods: Over a 2 year recruitment period, subjects with CF aged 7\u201318 performed pulmonary function tests (PFTs). Moreover, the nutritional and microbiological status, frequency of pulmonary exacerbations (PExs), and patients\u2019 health-related quality of life (HRQoL) were assessed. Results: The mean age of the children (n = 69) was 14.09 \u00b1 3.26 years; F\/M 37\/32. Spirometry-based diagnoses of normal lung function (forced expiratory volume in 1 s, FEV1 \u2265 90%pred), mild (FEV1 70\u201389%pred) and moderate (FEV1 40\u201369%pred) lung diseases were established in 34 (49.3%), 25 (36.2%), and 10 (14.5%) patients, respectively. An elevated lung clearance index (LCI &gt; 6.98) was observed in 85% of the subjects with normal FEV1. The presence of Pseudomonas aeruginosa infection (n = 16) and the number of PExs treated with IV antibiotics were associated with significantly worse PFT results. Conclusions: MBNW and IOS are more helpful tools than conventional techniques in assessing early lung disease in CF. LCI is a more useful parameter for detecting functional abnormalities than FEV1 in school-age children.<\/jats:p>","DOI":"10.3390\/jcm12144735","type":"journal-article","created":{"date-parts":[[2023,7,18]],"date-time":"2023-07-18T01:46:02Z","timestamp":1689644762000},"page":"4735","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Pulmonary Function Tests in the Evaluation of Early Lung Disease in Cystic Fibrosis"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6496-8737","authenticated-orcid":false,"given":"Katarzyna","family":"Walicka-Serzysko","sequence":"first","affiliation":[{"name":"Cystic Fibrosis Department, Institute of Mother and Child, 01-211 Warsaw, Poland"},{"name":"Cystic Fibrosis Centre, Paediatric Hospital, Dziekanow Lesny, 05-092 \u0141omianki, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdalena","family":"Postek","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Department, Institute of Mother and Child, 01-211 Warsaw, Poland"},{"name":"Cystic Fibrosis Centre, Paediatric Hospital, Dziekanow Lesny, 05-092 \u0141omianki, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Urszula","family":"Borawska-Kowalczyk","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Department, Institute of Mother and Child, 01-211 Warsaw, Poland"},{"name":"Cystic Fibrosis Centre, Paediatric Hospital, Dziekanow Lesny, 05-092 \u0141omianki, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justyna","family":"Milczewska","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Department, Institute of Mother and Child, 01-211 Warsaw, Poland"},{"name":"Cystic Fibrosis Centre, Paediatric Hospital, Dziekanow Lesny, 05-092 \u0141omianki, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorota","family":"Sands","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Department, Institute of Mother and Child, 01-211 Warsaw, Poland"},{"name":"Cystic Fibrosis Centre, Paediatric Hospital, Dziekanow Lesny, 05-092 \u0141omianki, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,7,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"eaav3488","DOI":"10.1126\/scitranslmed.aav3488","article-title":"Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis","volume":"11","author":"Esther","year":"2019","journal-title":"Sci. Transl. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1567","DOI":"10.1164\/rccm.201606-1107CI","article-title":"Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis. What Have We Learned and What Should We Do about It?","volume":"195","author":"Ranganathan","year":"2017","journal-title":"Am. J. Respir. Crit.Care Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1136\/thoraxjnl-2013-204023","article-title":"Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants","volume":"69","author":"Nguyen","year":"2014","journal-title":"Thorax"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1097\/MCP.0000000000000209","article-title":"Evolution of cystic fibrosis lung function in the early years","volume":"21","author":"Bush","year":"2015","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1255","DOI":"10.1136\/thoraxjnl-2020-216085","article-title":"Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis","volume":"76","author":"Bayfield","year":"2021","journal-title":"Thorax"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1186\/1465-9921-7-138","article-title":"Progression of pulmonary hyperinflation and trapped gas associated with genetic and environmental factors in children with cystic fibrosis","volume":"7","author":"Kraemer","year":"2006","journal-title":"Respir. Res."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/S2213-2600(19)30337-6","article-title":"The future of cystic fibrosis care: A global perspective","volume":"8","author":"Bell","year":"2020","journal-title":"Lancet Respir. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1080\/17476348.2018.1519400","article-title":"Predicting disease progression in cystic fibrosis","volume":"12","author":"Breuer","year":"2018","journal-title":"Expert Rev. Respir. Med."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1002\/ppul.21154","article-title":"Cystic fibrosis lung disease starts in the small airways: Can we treat it more effectively?","volume":"45","author":"Tiddens","year":"2010","journal-title":"Pediatr. Pulmonol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1164\/rccm.200407-895OC","article-title":"Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis","volume":"171","author":"Aurora","year":"2005","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.jcf.2013.09.005","article-title":"Lung clearance index: Evidence for use in clinical trials in cystic fibrosis","volume":"13","author":"Kent","year":"2014","journal-title":"J. Cyst. Fibros."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1164\/rccm.201507-1409OC","article-title":"Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic Fibrosis","volume":"193","author":"Ramsey","year":"2016","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1136\/thoraxjnl-2021-216928","article-title":"Longitudinal assessment of lung clearance index to monitor disease progression in children and adults with cystic fibrosis","volume":"77","author":"Horsley","year":"2022","journal-title":"Thorax"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"410","DOI":"10.4103\/0970-2113.184875","article-title":"Impulse oscillometry: The state-of-art for lung function testing","volume":"33","author":"Desiraju","year":"2016","journal-title":"Lung India"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1183\/09031936.00003407","article-title":"Respiratory impedance in children with cystic fibrosis using forced oscillations in clinic","volume":"30","author":"Gangell","year":"2007","journal-title":"Eur. Respir. J."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1002\/ppul.24286","article-title":"Identifying pediatric lung disease: A comparison of forced oscillation technique outcomes","volume":"54","author":"Evans","year":"2019","journal-title":"Pediatr. Pulmonol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1672","DOI":"10.1183\/13993003.00156-2015","article-title":"Impact of lung disease on respiratory impedance in young children with cystic fibrosis","volume":"46","author":"Ramsey","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1164\/rccm.202010-3899ED","article-title":"Defining the Clinical Utility of the Lung Clearance Index. Are We There Yet?","volume":"203","author":"Spano","year":"2021","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/j.jcf.2018.02.006","article-title":"ECFS best practice guidelines: The 2018 revision","volume":"17","author":"Castellani","year":"2018","journal-title":"J. Cyst. Fibros."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"S4","DOI":"10.1016\/j.jpeds.2008.05.005","article-title":"Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic fibrosis foundation consensus report","volume":"153","author":"Farrell","year":"2008","journal-title":"J. Pediatr."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1136\/thx.2005.043539","article-title":"Cystic fibrosis: Terminology and diagnostic algorithms","volume":"61","author":"Wilschanski","year":"2006","journal-title":"Thorax"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1304","DOI":"10.1164\/rccm.200605-642ST","article-title":"An official American Thoracic Society\/European Respiratory Society statement: Pulmonary function testing in preschool children","volume":"175","author":"Beydon","year":"2007","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1183\/09031936.05.00034805","article-title":"Standardisation of spirometry","volume":"26","author":"Miller","year":"2005","journal-title":"Eur. Respir. J."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1183\/09031936.00069712","article-title":"Consensus statement for inert gas washout measurement using multiple- and single-breath tests","volume":"41","author":"Robinson","year":"2013","journal-title":"Eur. Respir. J."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1900753","DOI":"10.1183\/13993003.00753-2019","article-title":"Technical standards for respiratory oscillometry","volume":"55","author":"King","year":"2020","journal-title":"Eur. Respir. J."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"2347","DOI":"10.1378\/chest.128.4.2347","article-title":"Development and validation of The Cystic Fibrosis Questionnaire in the United States: A health-related quality-of-life measure for cystic fibrosis","volume":"128","author":"Quittner","year":"2005","journal-title":"Chest"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1016\/S0031-3939(09)70151-9","article-title":"Polska adaptacja Kwestionariusza Jako\u015bci \u017bycia przeznaczonego dla dzieci i doros\u0142ych chorych na mukowiscydoz\u0119 oraz ich rodzic\u00f3w (CFQ-R)","volume":"84","author":"Sands","year":"2009","journal-title":"Pediatr. Polska."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1610","DOI":"10.1378\/chest.08-1190","article-title":"Determination of the Minimal Clinically Important Difference Scores for the Cystic Fibrosis Questionnaire-Revised Respiratory Symptom Scale in Two Populations of Patients with Cystic Fibrosis and Chronic Pseudomonas aeruginosa Airway Infection","volume":"135","author":"Quittner","year":"2009","journal-title":"Chest"},{"key":"ref_29","first-page":"369","article-title":"Silent lung zone\u2014Application of multi-breath nitrogen washout test (MBNW) in the diagnosis of small airways diseases in children\u2014Preliminary report based on literature and own experience","volume":"21","author":"Postek","year":"2017","journal-title":"Dev. Period Med."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1068","DOI":"10.1136\/thx.2004.022590","article-title":"Multiple breath inert gas washout as a measure of ventilation distribution in children with cystic fibrosis","volume":"59","author":"Aurora","year":"2004","journal-title":"Thorax"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1216","DOI":"10.1164\/rccm.201610-2158OC","article-title":"Progression of Lung Disease in Preschool Patients with Cystic Fibrosis","volume":"195","author":"Stanojevic","year":"2017","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1136\/thoraxjnl-2017-210979","article-title":"Lung clearance index to monitor treatment response in pulmonary exacerbations in preschool children with cystic fibrosis","volume":"73","author":"Rayment","year":"2018","journal-title":"Thorax"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1002\/ppul.24278","article-title":"Change in lung clearance index with microbiological status in children with cystic fibrosis","volume":"54","author":"Postek","year":"2019","journal-title":"Pediatr. Pulmonol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"3030","DOI":"10.1002\/ppul.25013","article-title":"Lung function deterioration in school children with cystic fibrosis","volume":"55","author":"Postek","year":"2020","journal-title":"Pediatr. Pulmonol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1136\/thx.2011.161117","article-title":"Exacerbation frequency and clinical outcomes in adult patients with cystic fibrosis","volume":"66","author":"Vandemheen","year":"2011","journal-title":"Thorax"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"S200","DOI":"10.1513\/AnnalsATS.201502-098AW","article-title":"Pulmonary Exacerbations in Children with Cystic Fibrosis","volume":"12","author":"Waters","year":"2015","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1164\/rccm.200909-1421OC","article-title":"Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation","volume":"182","author":"Sanders","year":"2010","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1002\/ppul.21374","article-title":"Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis","volume":"46","author":"Sanders","year":"2011","journal-title":"Pediatr. Pulmonol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1183\/09031936.00159111","article-title":"Effect of pulmonary exacerbations on long-term lung function decline in cystic fibrosis","volume":"40","author":"Waters","year":"2012","journal-title":"Eur. Respir. J."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1136\/thoraxjnl-2014-206750","article-title":"Short-term and long-term response to pulmonary exacerbation treatment in cystic fibrosis","volume":"71","author":"Heltshe","year":"2016","journal-title":"Thorax"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1002\/ppul.21117","article-title":"Return of FEV1 after pulmonary exacerbation in children with cystic fibrosis","volume":"45","author":"Sanders","year":"2010","journal-title":"Pediatr. Pulmonol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"977","DOI":"10.1164\/rccm.202006-2433OC","article-title":"Lung Clearance Index to Track Acute Respiratory Events in School-Age Children with Cystic Fibrosis","volume":"203","author":"Perrem","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1016\/j.jcf.2021.02.012","article-title":"The effect of oral and intravenous antimicrobials on pulmonary exacerbation recovery in cystic fibrosis","volume":"20","author":"VanDevanter","year":"2021","journal-title":"J. Cyst. Fibros."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1136\/thoraxjnl-2021-218347","article-title":"Evaluation of clinically relevant changes in the lung clearance index in children with cystic fibrosis and healthy controls","volume":"78","author":"Lucy","year":"2023","journal-title":"Thorax"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"2003380","DOI":"10.1183\/13993003.03380-2020","article-title":"Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis","volume":"58","author":"Stanojevic","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1466","DOI":"10.1002\/ppul.25891","article-title":"Predictive value of impulse oscillometry and multiple breath washout parameters in pediatric patients with cystic fibrosis pulmonary exacerbation","volume":"57","author":"Stachowiak","year":"2022","journal-title":"Pediatr. Pulmonol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1151","DOI":"10.1002\/ppul.23449","article-title":"Evaluation of children with cystic fibrosis by impulse oscillometry when stable and at exacerbation","volume":"51","author":"Sakarya","year":"2016","journal-title":"Pediatr. Pulmonol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1016\/j.jcf.2020.02.022","article-title":"Health-related quality-of-life in children with cystic fibrosis aged 5-years and associations with health outcomes","volume":"19","author":"Cheney","year":"2020","journal-title":"J. Cyst. Fibros."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1007\/s11136-011-0036-z","article-title":"Psychometric evaluation of the Cystic Fibrosis Questionnaire-Revised in a national sample","volume":"21","author":"Quittner","year":"2012","journal-title":"Qual. Life Res."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1002\/ppul.21325","article-title":"Longitudinal assessment of health-related quality of life in an observational cohort of patients with cystic fibrosis","volume":"46","author":"Sawicki","year":"2010","journal-title":"Pediatr. Pulmonol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1007\/s00431-017-2928-6","article-title":"Factors associated with changes in health-related quality of life in children with cystic fibrosis during 1-year follow-up","volume":"176","author":"Winkens","year":"2017","journal-title":"Eur. J. Pediatr."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1136\/thoraxjnl-2013-203807","article-title":"Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis","volume":"69","author":"Vermeulen","year":"2014","journal-title":"Thorax"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1016\/j.chest.2016.06.029","article-title":"Lung Clearance Index in Adults and Children with Cystic Fibrosis","volume":"150","author":"Tunney","year":"2016","journal-title":"Chest"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"e188","DOI":"10.1016\/j.jcf.2021.11.005","article-title":"Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis","volume":"21","author":"Pattie","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1016\/j.jcf.2020.02.017","article-title":"Changes in the parent cystic fibrosis questionnaire-revised (CFQ-R) with respiratory symptoms in preschool children with cystic fibrosis","volume":"19","author":"Perrem","year":"2020","journal-title":"J. Cyst. 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