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Table 2 Patient characteristics at last follow-up

From: Primary ciliary dyskinesia: critical evaluation of clinical symptoms and diagnosis in patients with normal and abnormal ultrastructure

  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
  1. Legend: Table 2 shows clinical status at last follow-up in the total patient group and in the 3 subgroups. P-values for the comparison between the three subgroups (Kruskal-Wallis for continuous variables and χ2 for proportions) are shown in the last column. * marks results that are significantly different from the reference population. For FEV1 and FVC the total number of patients was only 112 because some patients were too young (<6 years) to perform spirometry (n = 25) or because spirometry results were not available.
  2. BMI, body mass index.
  3. FEV 1 , forced expiratory volume in one second.
  4. FVC, forced vital capacity.
  5. IQR, inter quartile range.
  6. SD, standard deviation.