CYSTIC FIBROSIS

Lung clearance index is a sensitive, repeatable and practical measure of airways disease in adults with cystic fibrosis

A R Horsley^1,5, P M Gustafsson², K A Macleod^3,5, C Saunders^4,5, A P Greening^1,5, D J Porteous^1,5, J C Davies^4,5, S Cunningham^3,5, E W F W Alton^4,5, J A Innes^1,5

¹ School of Clinical and Molecular Medicine, University of Edinburgh, Edinburgh, UK
² Queen Silvia Children’s Hospital, Gothenburg, Sweden
³ Royal Hospital for Sick Children, Edinburgh, UK
⁴ Department of Gene Therapy, National Heart and Lung Institute, Imperial College, London, UK
⁵ UK Cystic Fibrosis Gene Therapy Consortium, UK

Dr A R Horsley, Medical Genetics, Molecular Medicine Centre, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK; alex.horsley{at}ed.ac.uk

Background: Lung clearance index (LCI) is a sensitive marker of early lung disease in children but has not been assessed in adults. Measurement is hindered by the complexity of the equipment required. The aims of this study were to assess performance of a novel gas analyser (Innocor) and to use it as a clinical tool for the measurement of LCI in cystic fibrosis (CF).

Methods: LCI was measured in 48 healthy adults, 12 healthy school-age children and 33 adults with CF by performing an inert gas washout from 0.2% sulfur hexafluoride (SF₆). SF₆ signal:noise ratio and 10–90% rise time of Innocor were compared with a mass spectrometer used in similar studies in children.

Results: Compared with the mass spectrometer, Innocor had a superior signal:noise ratio but a slower rise time (150 ms vs 60 ms) which may limit its use in very young children. Mean (SD) LCI in healthy adults was significantly different from that in patients with CF: 6.7 (0.4) vs 13.1 (3.8), p<0.001. Ten of the patients with CF had forced expiratory volume in 1 s 80% predicted but only one had a normal LCI. LCI repeats were reproducible in all three groups of subjects (mean intra-visit coefficient of variation ranged from 3.6% to 5.4%).

Conclusions: Innocor can be adapted to measure LCI and affords a simpler alternative to a mass spectrometer. LCI is raised in adults with CF with normal spirometry, and may prove to be a more sensitive marker of the effects of treatment in this group.

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