title: Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function.
abstract: BACKGROUND AND AIMS Sensitive outcome measures to assess the efficacy of therapeutic interventions in patients with cystic fibrosis (CF) with mild lung disease are currently lacking. Our objective was to study the ability of the lung clearance index (LCI), a measure of ventilation inhomogeneity, to detect a treatment response to hypertonic saline inhalation in paediatric patients with CF with normal spirometry. METHODS In a crossover trial, 20 patients with CF received 4 weeks of hypertonic saline (HS) and isotonic saline (IS) in a randomised sequence separated by a 4 week washout period. The primary end point was the change in the LCI due to HS versus IS. RESULTS Baseline characteristics including the LCI were not significantly different between both study periods. Four weeks of twice-daily HS inhalation significantly improved the LCI compared with IS (1.16, 95% CI 0.26 to 2.05; p=0.016), whereas other outcome measures such as spirometry and quality of life failed to reach statistical significance. Randomisation order had no significant impact on the treatment effect. CONCLUSIONS The LCI, but not spirometry was able to detect a treatment effect from HS inhalation in patients with CF with mild disease and may be a suitable tool to assess early intervention strategies in this patient population. Clinical trial number NCT00635141.
authors.affiliation: Hospital for Sick Children, 555 University Avenue, Toronto Ontario M5G 1X8, Canada. reshma.amin@sickkids.ca
authors.forename: Reshma
authors.initials: R
authors.lastname: Amin
authors.forename: Padmaja
authors.initials: P
authors.lastname: Subbarao
authors.forename: Alisha
authors.initials: A
authors.lastname: Jabar
authors.forename: Susan
authors.initials: S
authors.lastname: Balkovec
authors.forename: Renee
authors.initials: R
authors.lastname: Jensen
authors.forename: Shawn
authors.initials: S
authors.lastname: Kerrigan
authors.forename: Per
authors.initials: P
authors.lastname: Gustafsson
authors.forename: Felix
authors.initials: F
authors.lastname: Ratjen
mesh_terms: D000280:Administration, Inhalation; D000293:Adolescent; D002648:Child; D018592:Cross-Over Studies; D003550:Cystic Fibrosis / Q000503:physiopathology / Q000628:therapy*; D005541:Forced Expiratory Volume; D006801:Humans; D008168:Lung / Q000378:metabolism / Q000503:physiopathology; D009079:Mucociliary Clearance; D009330:Nebulizers and Vaporizers; D012462:Saline Solution, Hypertonic / Q000008:administration & dosage / Q000627:therapeutic use*; D012965:Sodium Chloride / Q000008:administration & dosage / Q000627:therapeutic use; D013147:Spirometry / Q000379:methods; D016896:Treatment Outcome
chemical_list: D012462:Saline Solution, Hypertonic; D012965:Sodium Chloride