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

Kieninger, Elisabeth; Yammine, Sophie; Korten, Insa Christina Severine; Anagnostopoulou, Pinelopi; Singer, Florian; Frey, Urs Peter; Mornand, Anne; Zanolari, Maura; Rochat, Isabelle; Trachsel, Daniel; Müller-Suter, Dominik; Moeller, Alexander; Casaulta, Carmen; Latzin, Philipp (2017). Elevated lung clearance index in infants with cystic fibrosis shortly after birth. European respiratory journal, 50(5) European Respiratory Society 10.1183/13993003.00580-2017

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Pneumology

Graduate School:

Graduate School for Cellular and Biomedical Sciences (GCB)

UniBE Contributor:

Kieninger, Elisabeth; Yammine, Sophie; Korten, Insa Christina Severine; Anagnostopoulou, Pinelopi; Singer, Florian; Frey, Urs Peter; Müller-Suter, Dominik; Casaulta, Carmen and Latzin, Philipp

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0903-1936

Publisher:

European Respiratory Society

Language:

English

Submitter:

Romy Melanie Rodriguez Del Rio

Date Deposited:

12 Feb 2018 11:08

Last Modified:

09 Jan 2019 10:48

Publisher DOI:

10.1183/13993003.00580-2017

PubMed ID:

29122915

BORIS DOI:

10.7892/boris.109241

URI:

https://boris.unibe.ch/id/eprint/109241

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