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

Research output: Contribution to journalArticlepeer-review

6 Citations (Scopus)
20 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

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