title: Long-term improvement of lung clearance index in patients with mild cystic fibrosis lung disease: Does hypertonic saline play a role?
abstract: To assess whether long-term inhalation with hypertonic saline is able to halt the progression of mild CF lung disease, we analysed longitudinal data of lung clearance index (LCI) and spirometry. A total of 34 patients with mild lung disease (FEV1 ≥ 70% of predicted) had at least one LCI result before and ≥2 LCI measurements after start of hypertonic saline (HS) therapy. After a mean follow-up of 39.7 (SD 7.4) months after starting HS, LCI improved significantly from 7.89 (SD 1.35) at baseline to 6.96 (SD 1.03), and 19/34 patients had a normal LCI value at the last measurement. No decrease in mean FEV1 was observed. Thus, ventilation inhomogeneity can improve in patients with mild lung disease.
authors.affiliation: Medical University of Innsbruck, Department of Child and Adolescent Health, Division of Cardiology, Pulmonology, Allergology, and Cystic Fibrosis, Cystic Fibrosis Centre, Anichstrasse 35, A-6020 Innsbruck, Austria. Electronic address: helmut.ellemunter@i-med.ac.at.
authors.forename: Helmut
authors.initials: H
authors.lastname: Ellemunter
authors.affiliation: Medical University of Innsbruck, Department of Child and Adolescent Health, Division of Cardiology, Pulmonology, Allergology, and Cystic Fibrosis, Cystic Fibrosis Centre, Anichstrasse 35, A-6020 Innsbruck, Austria.
authors.forename: Johannes
authors.initials: J
authors.lastname: Eder
authors.affiliation: Children's Hospital and Research Institute, Marien Hospital Wesel, Pastor Janßen Straße 8-38, 46483 Wesel, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Fuchs
authors.affiliation: Children's Hospital and Research Institute, Marien-Hospital Wesel, Marien-Hospital Wesel gGmbH, Pastor-Janßen-Str. 8-38, 46483 Wesel, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Gappa
authors.affiliation: Clinical Research, Reutzstr. 1, 19055 Schwerin, Germany.
authors.forename: Gratiana
authors.initials: G
authors.lastname: Steinkamp
mesh_terms: D000280:Administration, Inhalation; D000293:Adolescent; D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D065106:Demulcents / Q000008:administration & dosage; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D009079:Mucociliary Clearance / Q000187:drug effects*; D062072:Patient Acuity; D012129:Respiratory Function Tests / Q000379:methods; D012462:Saline Solution, Hypertonic / Q000008:administration & dosage*; D013995:Time; D016896:Treatment Outcome
chemical_list: D065106:Demulcents; D012462:Saline Solution, Hypertonic