Table 1

Demographic and diagnostic characteristics of study population

 Female/male (n)17/10
 Age, years11.3 (6.3–18.5)
 Age at diagnosis, years2.2 (0.04–9.8)
 Weight, kg36.5 (16.1–61.6)
 Height, cm145 (114–182)
Bacterial colonisation (n)
 Chronic: P aeruginosa1
 Intermittent: P aeruginosa2
 Intermittent: A xylosoxidans1
Diagnostic tests (n)
 Typical clinical symptoms27
 Nasal nitric oxide performed26
  Low level26
 Ciliary beat pattern and frequency analysis performed27
  Normal frequency, asynchrony6*
  Low frequency, asynchrony10*
 Electron microscopy performed21
 Ultrastructural defect
  Outer dynein arm (ODA)6
  Inner dynein arm (IDA)1
  Radial spoke defect + IDA5
  Transposition defect1
  Periphery microtubuli defect5
  • Demographic data were not normally distributed so are presented as median (range). Diagnostic characteristics are presented as n.

  • * Sum >25 as each patient can have more than one functional abnormality.