Characteristics of EPIC-PCD participants, overall and by age group (n=384)
| Total | Age 0–6 years | Age 7–14 years | Age 15–30 years | Age 31–50 years | Age >50 years | p-value# | |
| Participants | 384 (100) | 44 (100) | 122 (100) | 153 (100) | 42 (100) | 23 (100) | |
| Age years | 16 (9–22) | 4 (2–5) | 10 (8–12) | 18 (16–21) | 38 (34–43) | 57 (56–62) | |
| Male sex | 206 (54) | 23 (52) | 69 (57) | 79 (52) | 24 (57) | 11 (48) | 0.875 |
| Age at PCD diagnosis | 9 (4–17) | 1 (1–2) | 6 (2–8) | 13 (9–17) | 34 (29–36) | 51 (43–55) | |
| Consanguinity | 0.001 | ||||||
| Yes | 115 (30) | 7 (15) | 44 (36) | 49 (32) | 11 (26) | 4 (17) | |
| No | 130 (34) | 13 (30) | 35 (29) | 60 (39) | 19 (45) | 3 (13) | |
| Not reported | 139 (36) | 24 (55) | 43 (35) | 44 (29) | 12 (29) | 16 (70) | |
| Situs | <0.001 | ||||||
| Situs inversus totalis | 134 (35) | 25 (57) | 42 (34) | 56 (37) | 7 (17) | 4 (17) | |
| Situs ambiguous | 4 (1) | 0 (0) | 1 (1) | 3 (2) | 0 (0) | 0 (0) | |
| Situs solitus | 242 (63) | 18 (41) | 79 (65) | 94 (61) | 32 (76) | 19 (83) | |
| Not reported | 4 (1) | 1 (2) | 0 (0) | 0 (0) | 3 (7) | 0 (0) | |
| Cardiovascular malformation present | 0.093 | ||||||
| Yes | 33 (9) | 7 (16) | 10 (8) | 14 (9) | 2 (5) | 0 (0) | |
| No | 285 (74) | 30 (68) | 98 (80) | 110 (72) | 32 (76) | 15 (65) | |
| Not reported | 66 (17) | 7 (16) | 14 (12) | 29 (19) | 8 (19) | 8 (35) | |
| Neonatal rhinitis | 0.145 | ||||||
| Yes | 123 (32) | 15 (34) | 46 (38) | 48 (31) | 12 (29) | 2 (9) | |
| No | 83 (22) | 7 (16) | 23 (19) | 38 (25) | 11 (26) | 4 (17) | |
| Not reported | 178 (46) | 22 (50) | 53 (43) | 67 (44) | 19 (45) | 17 (74) | |
| Neonatal cough | 0.001 | ||||||
| Yes | 125 (33) | 15 (34) | 42 (34) | 57 (37) | 10 (24) | 1 (4) | |
| No | 86 (22) | 3 (7) | 28 (23) | 38 (25) | 14 (33) | 3 (13) | |
| Not reported | 173 (45) | 26 (59) | 52 (43) | 58 (38) | 18 (43) | 19 (83) | |
| Neonatal respiratory distress | 0.489 | ||||||
| Yes | 175 (46) | 26 (59) | 57 (46) | 20 (48) | 20 (47) | 8 (35) | |
| No | 139 (36) | 11 (25) | 46 (38) | 15 (36) | 15 (36) | 8 (35) | |
| Not reported | 70 (18) | 7 (16) | 19 (16) | 7 (17) | 7 (17) | 7 (30) | |
| Active smoking | <0.001 | ||||||
| Yes, daily | 4 (1) | NA | NA | 3 (2) | 0 (0) | 1 (4) | |
| Yes, rarely | 5 (1) | NA | NA | 3 (2) | 1 (2) | 1 (4) | |
| Ex-smoker | 15 (4) | NA | NA | 2 (1) | 8 (19) | 5 (22) | |
| Never-smoker | 183 (48) | NA | NA | 137 (90) | 31 (74) | 15 (66) | |
| Not reported | 177 (46) | NA | NA | 8 (5) | 2 (5) | 1 (4) | |
| Smoking in household | 0.001 | ||||||
| Yes | 66 (17) | 6 (14) | 22 (18) | 33 (22) | 3 (7) | 2 (9) | |
| No | 254 (66) | 34 (77) | 92 (75) | 86 (56) | 27 (64) | 15 (65) | |
| Not reported | 64 (17) | 4 (9) | 8 (7) | 34 (22) | 12 (29) | 6 (26) | |
Data are presented as n (%) or median (IQR). EPIC-PCD: Ear-nose throat Prospective International Cohort of patients with Primary Ciliary Dyskinesia; NA: not applicable (age-specific questionnaire version does not include question on active smoking in this age category). #: Chi-square test of independence.