Table 1.
Baseline characteristics | Study participants (n = 28) |
---|---|
Age in years, median (IQR) | 21.0 (16.0; 23.0) |
Female, n (%) | 11 (39.3) |
Pseudomonas aeruginosa positivea, n (%) | 15 (53.6) |
Staphylococcus aureus (MSSA) positiveb, n (%) | 16 (57.1) |
Staphylococcus aureus (MRSA) positiveb, n | 0 |
Stenotrophomonas maltophilia positiveb, n (%) | 1 (3.6) |
Burkholderia cepacia positiveb, n (%) | 1 (3.6) |
Body mass index, median (IQR) | 18.3 (16.2; 19.6) |
Pancreatic insufficient, n (%) | 26 (92.9) |
Diabetes mellitus, n (%) | 10 (35.7) |
ABPA, n (%) | 1 (3.6) |
ΔF508/ΔF508, n (%) | 17 (60.7) |
ΔF508 compound heterozygote, n (%) | 7 (25.0) |
Othersc, n (%) | 4 (14.3) |
FVC, litres, median (IQR) | 2.5 (1.6; 2.8) |
FVC % predicted, median (IQR) | 66.0 (47.8; 73.6) |
FEV1, litres, median (IQR) | 1.4 (0.9; 1.9) |
FEV1 % predicted, median (IQR) | 46.0 (43.0; 58.0) |
IQR interquartile range, MSSA, methicillin-susceptible S. aureus, MRSA methicillin-resistant S. aureus, ABPA allergic bronchopulmonary aspergillosis, FVC forced vital capacity, FEV forced expiratory volume in 1 second
a Pseudomonas aeruginosa positive is defined as two or more positive airway cultures in the previous year, currently receiving inhaled pseudomonal treatment, or both
bPositive is defined as two or more positive airway cultures in the previous year
cOthers is defined as mutations known to cause cystic fibrosis other than ΔF508 or not identified