Table 1 Lifetime prevalence of patient characteristics during follow-up
|  |  | 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 |
| Â | Male gender, n (%) | 91 (54.8) | 32 (54.2) | 39 (53.4) | 20 (58.8) | 0.85 |
| Â | North African ancestry, n (%) | 38 (23.3) | 8 (14.0) | 12 (16.7) | 18 (52.9)* | 0.0001 |
| Â | Consanguinity, n (%) | 33 (19.6) | 9 (15.3) | 12 (16.2) | 12 (34.3)* | 0.004 |
| Â | Sibling with PCD, n (%) | 37 (22.0) | 9 (15.3)* | 17 (23.0) | 11 (31.4)* | 0.047 |
| Â | Situs inversus, n (%) | 69 (41.1) | 18 (30.5) | 42 (56.8)* | 9 (25.7) | 0.001 |
| Â | 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 |
| Â | Wheezing, n (%) | 79 (47.0) | 28 (46.5) | 37 (50.0) | 14 (40.0) | 0.33 |
| Â | Bronchiectasis, n (%) | 114 (67.9) | 40 (67.8) | 51 (68.9) | 23 (65.7) | 0.51 |
| Â | Lobar collapse, n (%) | 68 (40.5) | 23 (39.0) | 30 (40.5) | 15 (42.9) | 0.16 |
| Â | Pulmonary infiltrate, n (%) | 100 (59.5) | 37 (62.7) | 43 (58.1) | 20 (57.1) | 0.29 |
| Â | Lobectomy, n (%) | 15 (8.9) | 4 (6.8) | 7(9.5) | 4 (11.4) | 0.49 |
| Â | H. influenzae, n (%) | 72 (42.9) | 28 (47.5) | 33 (44.6) | 11 (31.4) | 0.15 |
| Â | Strep. pneumoniae, n (%) | 45 (26.8) | 18 (30.5) | 20 (27.0) | 7 (20.0) | 0.08 |
| Â | Staph. aureus, n (%) | 28 (16.7) | 9 (15.3) | 17 (23.0) | 2 (5.7) | 0.07 |
| Â | 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 |
| Â | Nasal polyps, n (%) | 54 (32.1) | 19 (32.2) | 25 (33.8) | 10 (28.6) | 0.80 |
| Â | 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 |
| Â | Ear discharge, n (%) | 70 (41.7) | 30 (50.8) | 26 (35.1) | 14 (40.0) | 0.33 |
| Â | Ear drum perforation, n (%) | 35 (20.8) | 15 (25.4) | 11 (14.9) | 9 (25.7) | 0.15 |
| Â | Hearing loss, n (%) | 58 (34.5) | 20 (33.9) | 30 (40.5) | 8 (22.9) | 0.33 |
| Â | Hearing aid, n (%) | 10 (6.0) | 2 (3.4) | 5 (6.8) | 3 (8.6) | 0.38 |
| Â | Grommets insertion, n (%) | 82 (48.8) | 30 (50.8) | 33 (44.6) | 19 (54.3) | 0.56 |
| Â | Adenotomy, n (%) | 65 (38.7) | 28 (47.5) | 26 (35.1) | 11 (31.4) | 0.76 |