• Susanne I Fuchs, Monika Gappa, Johannes Eder, Karin M Unsinn, Gratiana Steinkamp, and Helmut Ellemunter.
• Department of Paediatrics and Research Institute, Marien-Hospital Wesel gGmbH, Pastor Janßen Straße 8-38, 46483 Wesel, Germany. Electronic address: susanne.fuchs@prohomine.de.
• Respir Med. 2014 Jun 1;108(6):865-74.

IntroductionLung disease remains the main cause of morbidity and mortality in patients with Cystic Fibrosis (CF). To detect lung disease before clinical symptoms become apparent, sensitive tools are essential. Spirometry is used for monitoring, but the FEV1 remains frequently normal throughout childhood. The Lung Clearance Index (LCI) calculated from Multiple Breath Washout (MBW) was introduced at the CF centre Innsbruck in 2007 for assessing ventilation inhomogeneity in patients with mild lung disease. We hypothesized that LCIs in 2007 are of prognostic value for the presence or absence of structural lung changes in later years.MethodsBetween 2007 and 2010 MBW, spirometry and ultra-low-dose HR-CT were prospectively tracked in 36 patients (6-53 years) with a mean FEV1 ≥ 80% predicted in 2007.ResultsAt study start the majority of patients had abnormal CT scores and LCI results. While CT and spirometry remained largely stable throughout the study, LCI results slightly improved but still correlated with CT scores in 2010. LCI results in 2007 correlated with CT scores in 2010 while FEV1 did not. In 86% the LCI value in 2007 was indicative for the presence or absence of structural lung changes in 2010.ConclusionThe LCI is a sensitive tool for detecting and tracking pulmonary changes. Extended structural changes are unlikely if the LCI is normal. The LCI has the potential to be used for monitoring the progression of early CF lung disease and assessing the effect of treatment in both clinical care and research settings.Copyright © 2014 Elsevier Ltd. All rights reserved.

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