Table 2.
Baseline Demographic and Clinical Characteristics by Ciliary Ultrastructural Defect Group
| Overall (n = 137) | ODA (n = 55) | ODA/IDA (n = 20) | IDA/CA/MTD (n = 41) | Normal EM (n = 12) | Other* (n = 9) | |
|---|---|---|---|---|---|---|
| Male, n (%) | 67 (49) | 27 (49) | 12 (60) | 21 (51) | 3 (25) | 4 (44) |
| Race, n (%) | ||||||
| White | 112 (82) | 48 (87) | 13 (65) | 32 (78) | 10 (83) | 9 (100) |
| Black | 3 (2) | — | 1 (5) | 2 (5) | — | — |
| Asian | 16 (12) | 4 (7) | 6 (30) | 4 (10) | 2 (17) | — |
| Native American | 1 (<1) | 1 (2) | — | — | — | — |
| Multiracial | 3 (2) | 2 (4) | — | 1 (2) | — | — |
| Not reported | 2 (2) | — | — | 2 (5) | — | — |
| Hispanic or Latino, n (%) | 13 (9) | 3 (5) | 1 (5) | 5 (12) | 2 (17) | 2 (22) |
| Age at initial diagnosis, yr, mean (SD) | 3.9 (3.8) | 4.8 (4.4) | 3.2 (3.4) | 2.5 (2.9)‡ | 5.2 (2.8) | 4.8 (3.1) |
| Age at enrollment, yr, mean (SD) | 7.8 (4.6) | 8.9 (4.3) | 7.5 (5.6) | 6.4 (4.4)‡ | 9.0 (4.4) | 7.1 (4.2) |
| Height percentile, mean (SD) | 47.9 (30.5) | 52.4 (31.4) | 44.7 (32.8) | 41.6 (25.8)‡ | 56.5 (35.3) | 38.4 (29.5) |
| Weight percentile, mean (SD) | 52.6 (31.2) | 60.8 (32.6) | 49.9 (34.7) | 40.4 (24.3)‡ | 54.4 (31.6) | 59.6 (29.5) |
| BMI percentile, mean (SD) | 55.4 (31.1) | 62.5 (30.7) | 50.2 (34.8) | 45.5 (26.6)‡ | 52.1 (33.9) | 65.3 (32.0) |
| FEV1 percent predicted (SD)† | 82.7 (19.1) | 88.3 (17.4) | 85.5 (21.4) | 71.5 (18.4)‡ | 86.1 (16.4) | 81.0 (9.5) |
| Nasal NO, nl/min, mean (SD) | 18.2 (20.1) | 14.0 (9.1) | 21.6 (24.2) | 17.8 (14.0) | 31.3 (48.4)‡ | 27.4 (21.1) |
| Clinical features, n (%) | ||||||
| Laterality defect | 71 (52) | 32 (58) | 10 (50) | 21 (51) | 8 (67) | 0 |
| Neonatal respiratory distress | 111 (81) | 43 (78) | 18 (90) | 36 (88) | 9 (75) | 5 (56) |
| Chronic cough | 136 (99) | 55 (100) | 20 (100) | 40 (98) | 12 (100) | 9 (100) |
| Chronic nasal congestion | 134 (98) | 54 (98) | 19 (95) | 40 (98) | 12 (100) | 9 (100) |
| Chronic otitis media | 127 (93) | 52 (95) | 18 (90) | 37 (90) | 11 (92) | 9 (100) |
Definition of abbreviations: BMI = body mass index; CA = central apparatus; EM = electron micrographs; IDA = inner dynein arm; MTD = microtubular disorganization; ODA = outer dynein arm.
*
Includes oligocilia (n = 4) and central apparatus defect (n = 5).
†
Baseline spirometry available from 92 participants; others were too young to perform spirometry at enrollment.
‡
P < 0.05 for comparison with ODA defect group.