Table 1 Characteristics of patients with PCD and CF
| Â | PCD | CF | p |
|---|---|---|---|
N | 20 | 20 | Â |
Male : Female | 12:8 | 13:7 | 1 |
Age (yrs) | 15.1 (8.7–29.4) | 16 (8–26) | 0.60 |
Age at diagnosis (yrs) | 9.9 (0.1–20.5) | 0.6 (0–16) | 0.03 |
Age at onset of respiratory symptoms (yrs) | 0.1 (0.1–4) | 0.6 (0–13) | 0.02 |
Duration of follow-up at tertiary center (yrs) | 6.9 (0.1–27.2) | 14.5 (0.1–25.9) | 0.009 |
Height (Z-score) | −0.71 (−2.55–1.81) | −0.47 (−2.41–1.5) | 0.88 |
Weight (Z-score) | 0.34 (−3.71–2.72) | −0.12 (−3.74–1.43) | 0.22 |
BMI (Z-score) | 0.77 (−2.84–2.7) | 0.39 (−2.53–1.88) | 0.13 |
Pancreatic insufficiency, n (%) | NA | 14 (70) | – |
Nasal nitric oxide (ppb) | 14 (5–54) | NA | – |
Situs viscerum inversus, n (%) | 12 (60) | NA | – |
Systemic antibiotic courses (previous 12 months) | 2 (0–7) | 2 (0–6) | 0.48 |
Hospital admissions (previous 12 months) | 0 (0–1) | 0 (0–5) | 0.38 |
FEV1 (Z-score) | −1.75 (−4.6–0.7) | −0.9 (−5.4–2.3) | 0.24 |
FVC (Z-score) | −0.6 (−3.9–1.8) | −0.3 (−3.4–2.5) | 0.37 |
FEV1/FVC (Z-score) | −1.6 (−3.5–0.1) | −1.1 (−4.2–1.1) | 0.24 |
FEF25–75 (Z-score) | −2 (−4.4–0.1) | −1.2 (−5.5–1.1) | 0.24 |
Sputum Microbiology | |||
 Chronic infection by P. aeruginosa | 1/20 | 3/20 | 0.60 |
 Chronic infection by H. influenzae | 6/20 | 2/20 | 0.23 |
  P. aeruginosa (≥1 sample) | 4/20 | 11/20 | 0.05 |
  H. influenzae (≥1 sample) | 15/20 | 11/20 | 0.32 |
  S. aureus (≥1 sample) | 3/20 | 13/20 | 0.003 |
Genetic analysis | |||
 ΔF508/ΔF508 | – | 6/20 |  |
 ΔF508/other | – | 10/20 |  |
 other/other | – | 4/20 |  |