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

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...

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Veröffentlicht in:The European respiratory journal 2017-11, Vol.50 (5), p.1700580-1700580
Hauptverfasser: Kieninger, Elisabeth, Yammine, Sophie, Korten, Insa, Anagnostopoulou, Pinelopi, Singer, Florian, Frey, Urs, Mornand, Anne, Zanolari, Maura, Rochat, Isabelle, Trachsel, Daniel, Mueller-Suter, Dominik, Moeller, Alexander, Casaulta, Carmen, Latzin, Philipp
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container_end_page 1700580
container_issue 5
container_start_page 1700580
container_title The European respiratory journal
container_volume 50
creator Kieninger, Elisabeth
Yammine, Sophie
Korten, Insa
Anagnostopoulou, Pinelopi
Singer, Florian
Frey, Urs
Mornand, Anne
Zanolari, Maura
Rochat, Isabelle
Trachsel, Daniel
Mueller-Suter, Dominik
Moeller, Alexander
Casaulta, Carmen
Latzin, Philipp
description 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
doi_str_mv 10.1183/13993003.00580-2017
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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&lt;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.</description><identifier>ISSN: 0903-1936</identifier><identifier>EISSN: 1399-3003</identifier><identifier>DOI: 10.1183/13993003.00580-2017</identifier><identifier>PMID: 29122915</identifier><language>eng</language><publisher>England: European Respiratory Society Journals Ltd</publisher><subject>Age ; Birth ; Breath Tests ; Case-Control Studies ; Children ; Cross-Sectional Studies ; Cystic fibrosis ; Cystic Fibrosis - diagnosis ; Cystic Fibrosis - physiopathology ; Female ; Functional Residual Capacity ; Humans ; Infant ; Infant, Newborn ; Infants ; Lung - physiopathology ; Lung diseases ; Male ; Medical screening ; Multivariate Analysis ; Neonatal Screening ; Prospective Studies ; Regression Analysis ; Respiratory function ; Switzerland</subject><ispartof>The European respiratory journal, 2017-11, Vol.50 (5), p.1700580-1700580</ispartof><rights>Copyright ©ERS 2017.</rights><rights>Copyright European Respiratory Society Journals Ltd. 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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&lt;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.</description><subject>Age</subject><subject>Birth</subject><subject>Breath Tests</subject><subject>Case-Control Studies</subject><subject>Children</subject><subject>Cross-Sectional Studies</subject><subject>Cystic fibrosis</subject><subject>Cystic Fibrosis - diagnosis</subject><subject>Cystic Fibrosis - physiopathology</subject><subject>Female</subject><subject>Functional Residual Capacity</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Infants</subject><subject>Lung - physiopathology</subject><subject>Lung diseases</subject><subject>Male</subject><subject>Medical screening</subject><subject>Multivariate Analysis</subject><subject>Neonatal Screening</subject><subject>Prospective Studies</subject><subject>Regression Analysis</subject><subject>Respiratory function</subject><subject>Switzerland</subject><issn>0903-1936</issn><issn>1399-3003</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkNtKAzEQhoMotlafQJCAN95sndnsIbmUUg9QEEGvl2w2sVu2uzXJqn17s7b1QpgDDP_8zHyEXCJMETm7RSYEA2BTgJRDFAPmR2Q8TKNhfEzGIIBFKFg2ImfOrQAwSxieklEsMA6ZjsnLvNGf0uuKNn37TlWjpZWt0rRuK_0daggjW-_oV-2XVG2drxU1dWk7Vzvqlp31zZZK47WlZW398pycGNk4fbHvE_J2P3-dPUaL54en2d0iUiznPpKJzlSsMM2qxAhRAiKoLE0rlmApMy6USirgBvLSVJwhZjFLY1PlRkMC4Y8Judn5bmz30Wvni3XtlG4a2equdwWKjMU5Chyk1_-kq663bbguqHjCeJwEYBPCdioVfnNWm2Jj67W02wKhGIgXB-LFL_FiIB62rvbefbnW1d_OATH7AV9Ueks</recordid><startdate>201711</startdate><enddate>201711</enddate><creator>Kieninger, Elisabeth</creator><creator>Yammine, Sophie</creator><creator>Korten, Insa</creator><creator>Anagnostopoulou, Pinelopi</creator><creator>Singer, Florian</creator><creator>Frey, Urs</creator><creator>Mornand, Anne</creator><creator>Zanolari, Maura</creator><creator>Rochat, Isabelle</creator><creator>Trachsel, Daniel</creator><creator>Mueller-Suter, Dominik</creator><creator>Moeller, Alexander</creator><creator>Casaulta, Carmen</creator><creator>Latzin, Philipp</creator><general>European Respiratory Society Journals Ltd</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>201711</creationdate><title>Elevated lung clearance index in infants with cystic fibrosis shortly after birth</title><author>Kieninger, Elisabeth ; 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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.</abstract><cop>England</cop><pub>European Respiratory Society Journals Ltd</pub><pmid>29122915</pmid><doi>10.1183/13993003.00580-2017</doi><tpages>1</tpages><oa>free_for_read</oa></addata></record>
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source MEDLINE; EZB Electronic Journals Library
subjects Age
Birth
Breath Tests
Case-Control Studies
Children
Cross-Sectional Studies
Cystic fibrosis
Cystic Fibrosis - diagnosis
Cystic Fibrosis - physiopathology
Female
Functional Residual Capacity
Humans
Infant
Infant, Newborn
Infants
Lung - physiopathology
Lung diseases
Male
Medical screening
Multivariate Analysis
Neonatal Screening
Prospective Studies
Regression Analysis
Respiratory function
Switzerland
title Elevated lung clearance index in infants with cystic fibrosis shortly after birth
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