Table 1 Patient characteristics
PCD (n = 32) | Bronchiectasis (n = 5) | p value | |
|---|---|---|---|
Female/male, no. (%) | 17/15 (53/47) | 5/0 (100/0) | 0.0673 |
Age in yrs, median (IQR) | 19 (15–28.5) | 68 (61–80) | 0.0001 |
Ultrastructural defect, n (%) | |||
Normal ultrastructure | 7/32 (22) | ||
ODA deficiency (complete or partial) | 14/32 (44) | ||
CP deficiency (complete or partial) | 8/32 (25) | ||
IDA deficiency (complete or partial) | 5/32 (16) | ||
Unknown | 2/32 (6) | ||
Genetic defect, n (%) | |||
DNAH5 | 7/32 (22) | ||
DNAH11 | 6/32 (19) | ||
CCDC40 | 4/32 (13) | ||
DNAAF1 | 2/32 (6) | ||
RSPH4 | 1/32 (3) | ||
RSPH9 | 1/32 (3) | ||
HYDIN | 1/32 (3) | ||
CCDC39 | 1/32 (3) | ||
CCDC65 | 1/32 (3) | ||
OFD1 | 1/32 (3) | ||
DNAL1 | 1/32 (3) | ||
Unknown | 6/32 (19) | ||
Clinical and laboratory features | |||
Bronchiectasis, n (%) | 30/32 (94) | 5/5 (100) | 0.9999 |
FEV1% of predicted, median (IQR) | 82 (66–93) | 85 (71–99.5) | 0.6026 |
FVC % of predicted, median (IQR) | 92 (82–102) | 108 (85.5–129.5) | 0.2070 |
CRP in mg/L, median (IQR) | 1.25 (0.23–4.13) | 3.20 (2.20–4.43) | 0.1905 |
Sputum bacteria, n (%) | |||
Haemophilus influenzae | 8/54 (15) | 0/5 (0) | |
Staphylococcus aureus | 11/54 (20) | 0/5 (0) | |
Pseudomonas aeruginosa | 10/54 (19) | 3/5 (60) | |
Streptococcus pneumoniae | 1/54 (2) | 0/5 (0) | |
Moraxella catarrhalis | 2/54 (4) | 0/5 (0) | |
Achromobacter xylosoxidans | 1/54 (2) | 0/5 (0) | |
Acetinobacter species | 1/54 (2) | 0/5 (0) | |
None | 28/54 (52) | 2/5 (40) | |
Unknown | 0/54 (0) | 2/5 (40) | |
Treatment, n (%) | |||
Azithromycin maintenance | 16/54 (30) | 1/5 (20) | |
Other oral antibiotics | 10/54 (19) | 0/5 (0) | |
Inhaled antibiotics | 3/54 (6) | 1/5 (20) | |
Inhaled steroids | 6/54 (11) | 3/5 (60) | |
Inhaled bronchodilators | 2/54 (4) | 1/5 (20) | |
Inhaled hypertonic saline | 10/54 (19) | 3/5 (60) |