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

SCILD and BILD study groups

doi:10.1183/13993003.00580-2017

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

SCILD and BILD study groups

Clinic of Pediatric and Adolescent Medicine

35 Citations (Scopus)

Abstract

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.

Original language	English
Article number	1700580
Journal	European Respiratory Journal
Volume	50
Issue number	5
ISSN	0903-1936
DOIs	https://doi.org/10.1183/13993003.00580-2017
Publication status	Published - 01.11.2017

Funding

Support statement: The work for this report was funded by the Swiss Society for Cystic Fibrosis (CFCH), the Gottfried und Julia Bangerter-Rhyner-Stiftung, the Fondazione Ettore e Valeria Rossi, the Swiss National Science Foundation (grant 144280 and 163311 to U. Frey, P. Latzin and C. Kuehni) and the Botnar Foundation. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Funding information for this article has been deposited with the Crossref Funder Registry.

Research Areas and Centers

Academic Focus: Center for Brain, Behavior and Metabolism (CBBM)

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1183/13993003.00580-2017

Cite this

@article{53b272160ab242d2a33472576fc845b1,

title = "Elevated lung clearance index in infants with cystic fibrosis shortly after birth",

abstract = "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.",

author = "\{SCILD and BILD study groups\} and Elisabeth Kieninger and Sophie Yammine and Insa Korten and Pinelopi Anagnostopoulou and Florian Singer and Urs Frey and Anne Mornand and Maura Zanolari and Isabelle Rochat and Daniel Trachsel and Dominik Mueller-Suter and Alexander Moeller and Carmen Casaulta and Philipp Latzin and Juerg Barben and Gaudenz Hafen and Nicolas Regamey and Kentgens, \{Anne Christianne\} and Barbara Egger and Oliver Fuchs and Claudia Kuehni and Elena Proietti and Anne Schmidt and Jakob Usemann",

note = "Funding Information: Support statement: The work for this report was funded by the Swiss Society for Cystic Fibrosis (CFCH), the Gottfried und Julia Bangerter-Rhyner-Stiftung, the Fondazione Ettore e Valeria Rossi, the Swiss National Science Foundation (grant 144280 and 163311 to U. Frey, P. Latzin and C. Kuehni) and the Botnar Foundation. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Funding information for this article has been deposited with the Crossref Funder Registry. Publisher Copyright: {\textcopyright} Copyright ERS 2017. Copyright: Copyright 2019 Elsevier B.V., All rights reserved.",

year = "2017",

month = nov,

day = "1",

doi = "10.1183/13993003.00580-2017",

language = "English",

volume = "50",

journal = "European Respiratory Journal",

issn = "0903-1936",

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T1 - Elevated lung clearance index in infants with cystic fibrosis shortly after birth

AU - SCILD and BILD study groups

AU - Kieninger, Elisabeth

AU - Yammine, Sophie

AU - Korten, Insa

AU - Anagnostopoulou, Pinelopi

AU - Singer, Florian

AU - Frey, Urs

AU - Mornand, Anne

AU - Zanolari, Maura

AU - Rochat, Isabelle

AU - Trachsel, Daniel

AU - Mueller-Suter, Dominik

AU - Moeller, Alexander

AU - Casaulta, Carmen

AU - Latzin, Philipp

AU - Barben, Juerg

AU - Hafen, Gaudenz

AU - Regamey, Nicolas

AU - Kentgens, Anne Christianne

AU - Egger, Barbara

AU - Fuchs, Oliver

AU - Kuehni, Claudia

AU - Proietti, Elena

AU - Schmidt, Anne

AU - Usemann, Jakob

N1 - Funding Information: Support statement: The work for this report was funded by the Swiss Society for Cystic Fibrosis (CFCH), the Gottfried und Julia Bangerter-Rhyner-Stiftung, the Fondazione Ettore e Valeria Rossi, the Swiss National Science Foundation (grant 144280 and 163311 to U. Frey, P. Latzin and C. Kuehni) and the Botnar Foundation. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Funding information for this article has been deposited with the Crossref Funder Registry. Publisher Copyright: © Copyright ERS 2017. Copyright: Copyright 2019 Elsevier B.V., All rights reserved.

PY - 2017/11/1

Y1 - 2017/11/1

N2 - 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.

AB - 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.

UR - https://www.scopus.com/pages/publications/85034989685

U2 - 10.1183/13993003.00580-2017

DO - 10.1183/13993003.00580-2017

M3 - Journal articles

C2 - 29122915

AN - SCOPUS:85034989685

SN - 0903-1936

VL - 50

JO - European Respiratory Journal

JF - European Respiratory Journal

IS - 5

M1 - 1700580

ER -

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

Abstract

Funding

Research Areas and Centers

UN SDGs

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