Table 1 Characteristics of patients reported as number and percentage or mean ± SD
| Â | Children | Adults |
|---|---|---|
Number of patients(%) | 21(36.2) | 37(63.8) |
PCD positive | 18(85.7) | 33(89.2) |
PCD highly likely | 3(14.3) | 4(10.8) |
Males(%) | 7(33.3) | 19(51.3) |
Age in years(mean ± SD) | 11.1 ± 4.6 | 39.4 ± 14.4 |
Age at diagnosis(mean ± SD) | 4.3 ± 4.2 | 26.5 ± 16.2 |
Situs inversus(%) | 14(66.6) | 22(59.4) |
Situs solitus(%) | 6(28.6) | 15(40.5) |
Situs ambiguous(%) | 1(4.8) | 0(0.0) |
Lobectomy(%) | 1(4.8) | 5(13.5) |
Percentage of predicted value | ||
FEV1(mean ± SD) | 93.3 ± 18.7 | 72.5 ± 23.4 |
FVC(mean ± SD) | 103.5 ± 15.6 | 85.2 ± 21.7 |
Exacerbations(mean ± SD) | 1.6 ± 1.0 | 2.3 ± 1.1 |
Exacerbations ≥ 2/year(%) | 10(47.6) | 26(70.3) |
Colonization by: | ||
Pseudomonas aeruginosa | 3(14.3) | 12(32.4) |
Other pathogens | 9(42.8) | 9(24.3) |
Chest CT score (mBhalla) | 2.6 ± 6.2 | 16.2 ± 9.5 |
Bronchiectasis(%) | 6(28.6) | 32(86.5) |
Lobes(mean ± SD) | 0.6 ± 1 | 2.5 ± 1.3 |
Severity of bronchiectasis | ||
BSI | 2.5 ± 3.1 | 5.1 ± 4.3 |
FACED | 0.4 ± 0.8 | 1.2 ± 1.2 |
eFACED | 0.8 ± 1.4 | 2.1 ± 2.0 |