Demographic and diagnostic characteristics of study population
| Demographics | |
| Number | 27 |
| Female/male (n) | 17/10 |
| Age, years | 11.3 (6.3–18.5) |
| Age at diagnosis, years | 2.2 (0.04–9.8) |
| Weight, kg | 36.5 (16.1–61.6) |
| Height, cm | 145 (114–182) |
| Bacterial colonisation (n) | |
| Chronic: P aeruginosa | 1 |
| Intermittent: P aeruginosa | 2 |
| Intermittent: A xylosoxidans | 1 |
| Diagnostic tests (n) | |
| Typical clinical symptoms | 27 |
| Nasal nitric oxide performed | 26 |
| Low level | 26 |
| Ciliary beat pattern and frequency analysis performed | 27 |
| Normal frequency, asynchrony | 6* |
| Low frequency, asynchrony | 10* |
| Immotility | 10* |
| Normal | 1* |
| Inconclusive | 2* |
| Electron microscopy performed | 21 |
| Ultrastructural defect | |
| Outer dynein arm (ODA) | 6 |
| Inner dynein arm (IDA) | 1 |
| ODA+IDA | 2 |
| Radial spoke defect + IDA | 5 |
| Transposition defect | 1 |
| Periphery microtubuli defect | 5 |
| Normal | 1 |
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.