Lung Clearance Index (LCI) is Stable in Most Primary Ciliary Dyskinesia (PCD) Patients Managed in a Specialist Centre: a Pilot Study

S. Irving, S. Carr, C. Hogg, M. Loebinger, A. Shoemark, A. Bush

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    10 Citations (Scopus)
    114 Downloads (Pure)

    Abstract

    Primary ciliary dyskinesia is a condition in which abnormal cilia structure or function leads to reduced mucociliary clearance and obstructive lung disease. Twenty-nine patients had lung clearance index (LCI) measured in 2009 and we attempted to perform a 5-year follow-up. Only 12 patients could be re-recruited, but in this small group LCI was stable over the 5 years, which confirms previous data showing that spirometry is also stable in these patients over the medium term. The two patients with the highest LCI in 2009 had since died, despite one having relatively preserved spirometry at the time. These data may be used to inform sample size calculations of future studies.

    Original languageEnglish
    Pages (from-to)441-443
    Number of pages3
    JournalLung
    Volume195
    Issue number4
    Early online date20 Jun 2017
    DOIs
    Publication statusPublished - 1 Aug 2017

    Keywords

    • Multiple breath washout
    • Paediatrics
    • Primary ciliary dyskinesia
    • Rare disease

    ASJC Scopus subject areas

    • Pulmonary and Respiratory Medicine

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