title: Long-term course of lung clearance index between infancy and school-age in cystic fibrosis subjects.
abstract: Multiple breath washout (MBW) measurements have recently been shown to be sensitive for detection of early cystic fibrosis (CF) lung disease, with the lung clearance index (LCI) being the most common measure for ventilation inhomogeneity. The aim of this observational study was to describe the longitudinal course of LCI from time of clinical diagnosis during infancy to school-age in eleven children with CF. Elevated LCI during infancy was present in seven subjects, especially in those with later clinical diagnosis. Tracking of LCI at follow-up was evident only in the four most severe cases. We provide the first longitudinal data describing the long-term course of LCI in a small group of infants with CF. Our findings support the clinical usefulness of MBW measurements to detect and monitor early lung disease in children with CF already present shortly after clinical diagnosis.
authors.affiliation: Division of Respiratory Medicine, Department of Paediatrics, University Children's Hospital of Bern, Inselspital, 3010 Bern, Switzerland.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Kieninger
authors.forename: Florian
authors.initials: F
authors.lastname: Singer
authors.forename: Oliver
authors.initials: O
authors.lastname: Fuchs
authors.forename: Chiara
authors.initials: C
authors.lastname: Abbas
authors.forename: Urs
authors.initials: U
authors.lastname: Frey
authors.forename: Nicolas
authors.initials: N
authors.lastname: Regamey
authors.forename: Carmen
authors.initials: C
authors.lastname: Casaulta
authors.forename: Philipp
authors.initials: P
authors.lastname: Latzin
mesh_terms: D002648:Child; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D013997:Time Factors