One-year evolution and variability in multiple-breath washout indices in children and young adults with primary ciliary dyskinesia.

ABSTRACT: Background and objective: Cross-sectional and longer-term studies have demonstrated abnormal yet stable multiple-breath inert gas washout (MBW) indices in patients with primary ciliary dyskinesia (PCD). This study aimed to assess the intermediate term evolution and the between-occasion variability of MBW indices in PCD over 1 year. Methods: Children and young adults with a confirmed diagnosis of PCD were included in this single-centre, prospective, observational, longitudinal study. Over 1 year, nitrogen (N₂) MBW and spirometry were performed at three occasions during ordinary scheduled outpatient visits. Trends and variability in lung clearance index (LCI), moment ratios, normalized N₂ concentration at six lung volume turnovers, and regional ventilation

SUBMITTER: Kobbernagel HE 

PROVIDER: S-EPMC6442098 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature