Table 2 Patient characteristics at last follow-up
|  | Total (n = 168) | Normal ultrastructure (n = 59) | Dynein deficiency (n = 74) | Central pair abnormalities (n = 35) | p-value for comparison between groups |
|---|---|---|---|---|---|
Age in years (median - IQR) | 17.7 (9.5–28.1) | 15.8 (9.7–26.7) | 19.6 (10.9–31.9) | 14.2 (6.7 – 24.5) | 0.14 |
Years since diagnosis (median – IQR) | 4.3 (0.0–11.4) | 4.3 (0.4–9.0) | 3.6 (0.0–12.8) | 5.3 (0.0–11.5) | 0.88 |
Weight z-score (mean - SD) | −0.12 (1.25) | −0.25 (1.25) | 0.07 (1.33) | −0.51 (0.97)* | 0.16 |
Height z-score (mean - SD) | −0.63 (1.23)* | −0.53 (1.14)* | −0.64 (1.26)* | −0.91 (1.32)* | 0.67 |
BMI z-score (mean - SD) | 0.15 (1.23) | 0.02 (1.17) | 0.41 (1.22) | −0.25 (1.31) | 0.10 |
Chronic cough, n (%) | 136 (81.0) | 48 (81.4) | 65 (87.8) | 23 (65.7) | 0.13 |
Chronic sputum production, n (%) | 113 (81.9) | 43 (81.1) | 52 (83.9) | 18 (78.3) | 0.83 |
Clubbing, n (%) | 31 (18.5) | 10 (16.9) | 16 (21.6) | 5 (14.3) | 0.57 |
Nasal secretions, n (%) | 136 (81) | 49 (83.1) | 60 (81.1) | 27 (77.1) | 0.63 |
FEV1 z-score (median - IQR) (n = 112) | −1.79 (−2.96; –0.76)* | −1.47 (−2.67; –0.47)* | −1.94 (−3.13; –1.19)* | −2.34 (−3.61; –0.55)* | 0.17 |
FVC z-score (median - IQR) (n = 112) | −0.76 (−1.61; –0.15)* | −0.54 (−1.43; 0.06)* | −0.98 (−1.77; –0.21)* | −0.77 (−2.05; –0.30)* | 0.75 |