Table 1.
Characteristics of EPIC-PCD participants, overall and by age group (n=404)
| Variable | Total | Age 0–6 yr | Age 7–14 yr | Age 15–30 yr | Age 31–50 yr | Age >50 yr | |
|---|---|---|---|---|---|---|---|
| Number of participants | 404 (100) | 45 (100) | 131 (100) | 163 (100) | 42 (100) | 23 (100) | |
| Age (yr) | 15 (9–22) | 4 (2–5) | 10 (8–12) | 18 (16–22) | 37 (34–42) | 57 (56–62) | |
| Female sex | 187 (46) | 21 (47) | 59 (45) | 77 (47) | 18 (43) | 12 (52) | |
| Age at PCD diagnosis (yr) | 9 (3–17) | 1 (0–2) | 6 (1–8) | 13 (8–17) | 34 (29–36) | 51 (43–55) | |
| Laterality defect | |||||||
| Situs inversus totalis | 144 (35) | 25 (56) | 46 (35) | 61 (37) | 8 (19) | 4 (17) | |
| Situs ambiguous | 4 (1) | 0 | 1 (1) | 3 (2) | 0 | 0 | |
| Situs solitus | 253 (63) | 19 (42) | 84 (64) | 99 (61) | 32 (76) | 19 (83) | |
| Not reported | 3 (1) | 1 (2) | 0 | 0 | 2 (5) | 0 | |
| Cardiovascular malformation | |||||||
| Yes | 35 (9) | 7 (16) | 11 (8) | 15 (9) | 2 (5) | 0 | |
| No | 303 (75) | 31 (68) | 106 (81) | 119 (73) | 32 (76) | 15 (65) | |
| Not reported | 66 (16) | 7 (16) | 14 (11) | 29 (18) | 8 (19) | 8 (35) | |
| Patient-/parent-reported symptoma) | |||||||
| Runny nose | 248 (61) | 30 (67) | 77 (59) | 98 (60) | 26 (62) | 17 (74) | |
| Blocked nose | 242 (60) | 18 (40) | 81 (62) | 100 (61) | 31 (74) | 12 (52) | |
| Headache while bending down | 44 (11) | 1 (2) | 7 (5) | 29 (18) | 5 (12) | 2 (9) | |
| Ear pain | 207 (51) | 15 (33) | 60 (46) | 83 (51) | 30 (71) | 19 (83) | |
| Ear discharge | 109 (27) | 8 (18) | 38 (29) | 43 (26) | 11 (26) | 9 (39) | |
| Hearing problems (n=280)b) | 133 (48) | 9 (3) | 34 (12) | 45 (16) | 27 (10) | 18 (6) | |
| Examination findings | |||||||
| Nasal discharge | 300 (74) | 30 (67) | 100 (76) | 118 (72) | 34 (81) | 18 (78) | |
| Nasal polypsc) | 55 (14) | 2 (4) | 11 (8) | 24 (15) | 13 (31) | 5 (22) | |
| Hypertrophic turbinatesc) | 222 (55) | 23 (51) | 65 (50) | 102 (63) | 20 (48) | 12 (52) | |
| Facial pain | 53 (13) | 0 | 9 (7) | 23 (14) | 12 (29) | 9 (39) | |
| Acute otitis media | 6 (1) | 3 (7) | 3 (2) | 0 | 0 | 0 | |
| Otitis media with effusion | 122 (30) | 17 (38) | 50 (38) | 43 (26) | 6 (14) | 6 (26) | |
| Ear discharge | 36 (9) | 2 (4) | 15 (11) | 15 (9) | 1 (2) | 3 (13) | |
| Hearing loss measured at audiometry (n=280)b),d) | 119 (43) | 10 (4) | 30 (11) | 35 (13) | 24 (9) | 20 (7) | |
Values are presented as number (%) or median (interquartile range).
EPIC-PCD, ENT Prospective International Cohort of Patients with Primary Ciliary Dyskinesia.
a)
Ever reported at any frequency during the past 3 months.
b)
All % refer to 280 participants with available audiometry results.
c)
Bilateral or unilateral.
d)
Hearing loss measured at audiometry ranging from mild to profound based on the World Health Organization grade–could be bilateral or unilateral.