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

Kieninger, Elisabeth and Yammine, Sophie and Korten, Insa and Anagnostopoulou, Pinelopi and Singer, Florian and Frey, Urs and Mornand, Anne and Zanolari, Maura and Rochat, Isabelle and Trachsel, Daniel and Mueller-Suter, Dominik and Moeller, Alexander and Casaulta, Carmen and Latzin, Philipp and Scild study group, and Bild study group, . (2017) Elevated lung clearance index in infants with cystic fibrosis shortly after birth. European Respiratory Journal, 50 (5). p. 1700580.

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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.
Faculties and Departments:03 Faculty of Medicine > Bereich Kinder- und Jugendheilkunde (Klinik) > Kinder- und Jugendheilkunde (UKBB) > Pädiatrie (Frey)
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Kinder- und Jugendheilkunde (Klinik) > Kinder- und Jugendheilkunde (UKBB) > Pädiatrie (Frey)
UniBasel Contributors:Frey, Urs Peter
Item Type:Article, refereed
Article Subtype:Research Article
Publisher:European Respiratory Society
Note:Publication type according to Uni Basel Research Database: Journal article
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Last Modified:03 Mar 2021 15:29
Deposited On:03 Mar 2021 11:35

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