Table 1.
Clinical characteristics of patients with PCD
| Age, yr | Sex | Race | Consanguineous Parents | Clinically Predominant Feature | CBF (Average/Median), Hz | Ultrastructural Defect | FVC, % | FEV1, % | FEF25–75, % |
|---|---|---|---|---|---|---|---|---|---|
| 1 | F | C | No | CHD + SI | 3.2/3 | RS | |||
| 7 | M | C | Yes | SI + Brq | 5.1/3 | RS | 89 | 78 | 60 |
| 7 | M | C | Yes | Brq | 7.2/8 | Transposition | 81 | 76 | 40 |
| 9 | F | C | Yes | Brq + URD | 3.6/3 | Transposition | 63 | 54 | 40 |
| 12 | M | C | Yes | Brq | 2.8/2 | RS | 126 | 86 | 40 |
| 12 | M | C | Yes | SI + Brq | 2.3/2 | Outer dynein arm | |||
| 13 | M | C | Yes | SI + Brq | 3.6/2 | Outer dynein arm | 111 | 99 | 90 |
| 14 | F | C | No | Brq + URD | 7.4/7 | Transposition | 113 | 100 | 98 |
| 15 | M | C | Yes | SI + Brq | 2.2/2 | RS | 42 | 21 | 9 |
| 19 | M | NC | Yes | Brq | 5.7/5 | RS | 88 | 73 | 41 |
| 19 | F | C | No | SI + Brq | 6.4/3 | Normal | 88 | 70 | 50 |
| 19 | F | C | No | SI + Brq | 2.3/2 | Shortened outer arms | 105 | 89 | 75 |
F, female; M, male; CHD, congenital heart disease; SI, situs inversus; Brq, bronchiectasis; URD, upper respiratory disease; CBF, ciliary beat frequency; C, Caucasian origin; NC, non-Caucasian origin; RS, radial spoke; FVC, forced vital capacity; FEV1, forced expiratory volume at the 1st s; FEF25–75, forced expiratory flow between 25 and 75% of expired vital capacity.