TABLE 2.
Variable | Mean (SD) or % |
---|---|
Genotype (F508del homozygous) | 56% |
CFTR function (mean sweat chloride, mmol/L) | 98 (13.1) |
iPFT | |
FVC% | 108 (17.2) |
FEV0.5% | 102 (19.9) |
FEF25–75% | 96 (28.5) |
RV/TLC% | 144 (24.6) |
Nutritional status (weight/length percentile at 12 months) |
65% (24%) |
Microbiology (history of PsA) | 44% |
Cough (occasional/daily) | 42% |
Pulmonary exacerbations (in first 12 months of life; range 1–5) |
1.9 (1.5) |
Hospitalizations (in first 12 months of life) | 47% |
Clinical score (range 0–10) | 7.6 (2.1) |
Prescribed chronic pulmonary therapies | 67% |
Inhaled corticosteroid | 42% |
Dornase alpha | 40% |
Azithromycin | 23% |