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Table 1 Lifetime prevalence of patient characteristics during follow-up

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

   Total population (n = 168) Normal ultrastructure (n = 59) Dynein deficiency (n = 74) Central pair abnormalities (n = 35) p-value for comparison between groups
General Age at diagnosis in years (median - IQR) 9.9 (3.7–23.4) 10.2 (4.4–21.8) 10.8 (3.7–29.6) 8.7 (2.2–19.3) 0.60
  Male gender, n (%) 91 (54.8) 32 (54.2) 39 (53.4) 20 (58.8) 0.85
  North African ancestry, n (%) 38 (23.3) 8 (14.0) 12 (16.7) 18 (52.9)* 0.0001
  Consanguinity, n (%) 33 (19.6) 9 (15.3) 12 (16.2) 12 (34.3)* 0.004
  Sibling with PCD, n (%) 37 (22.0) 9 (15.3)* 17 (23.0) 11 (31.4)* 0.047
  Situs inversus, n (%) 69 (41.1) 18 (30.5) 42 (56.8)* 9 (25.7) 0.001
  Structural cardiac abnormality, n(%) 11 (6.5) 4 (8.7) 5 (9.6) 2 (9.1) 0.75
Lower respiratory tract Neonatal respiratory problems, n (%) 75 (44.6) 27 (45.8) 30 (40.5) 18 (51.4) 0.60
  Wheezing, n (%) 79 (47.0) 28 (46.5) 37 (50.0) 14 (40.0) 0.33
  Bronchiectasis, n (%) 114 (67.9) 40 (67.8) 51 (68.9) 23 (65.7) 0.51
  Lobar collapse, n (%) 68 (40.5) 23 (39.0) 30 (40.5) 15 (42.9) 0.16
  Pulmonary infiltrate, n (%) 100 (59.5) 37 (62.7) 43 (58.1) 20 (57.1) 0.29
  Lobectomy, n (%) 15 (8.9) 4 (6.8) 7(9.5) 4 (11.4) 0.49
  H. influenzae, n (%) 72 (42.9) 28 (47.5) 33 (44.6) 11 (31.4) 0.15
  Strep. pneumoniae, n (%) 45 (26.8) 18 (30.5) 20 (27.0) 7 (20.0) 0.08
  Staph. aureus, n (%) 28 (16.7) 9 (15.3) 17 (23.0) 2 (5.7) 0.07
  Pseud. aeruginosa, n (%) 27 (16.1) 9 (15.3) 14 (18.9) 4 (11.4) 0.59
Upper respiratory tract Recurrent sinusitis, n (%) 111 (66.1) 41 (69.5) 52 (70.3) 18 (51.4) 0.67
  Nasal polyps, n (%) 54 (32.1) 19 (32.2) 25 (33.8) 10 (28.6) 0.80
  Sinus surgery, n (%) 68 (40.5) 27 (45.8) 31 (41.9) 10 (28.6) 0.79
  nNO (ppb) (median – IQR) 52 (30–170) 64 (37–199) 40 (25–98) 89 (24–327) 0.15
  Ear discharge, n (%) 70 (41.7) 30 (50.8) 26 (35.1) 14 (40.0) 0.33
  Ear drum perforation, n (%) 35 (20.8) 15 (25.4) 11 (14.9) 9 (25.7) 0.15
  Hearing loss, n (%) 58 (34.5) 20 (33.9) 30 (40.5) 8 (22.9) 0.33
  Hearing aid, n (%) 10 (6.0) 2 (3.4) 5 (6.8) 3 (8.6) 0.38
  Grommets insertion, n (%) 82 (48.8) 30 (50.8) 33 (44.6) 19 (54.3) 0.56
  Adenotomy, n (%) 65 (38.7) 28 (47.5) 26 (35.1) 11 (31.4) 0.76
  1. Legend: Table 1 shows general patient characteristics, incidence of specific lower and upper respiratory tract signs and symptoms in the total PCD 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. If they revealed a significant result, pairwise comparisons were performed and the significant outlier was identified and marked with *.
  2. IQR, inter quartile range.
  3. nNO, nasal nitric oxide.
  4. PCD, primary ciliary dyskinesia.
  5. Data marked in bold denote significant differences.