Table 1.
Subject Demographics
| Cystic Fibrosis | ||||
|---|---|---|---|---|
| Healthy | All | Heterozygous ΔF508 | Homozygous ΔF508 | |
| n | 16 | 14 | 5 | 9 |
| Gender male | 10 (63) | 12(86) | 4 (80) | 8 (89) |
| Age (years) | 24±6 | 22±8 | 26±9 | 20±7 |
| Height (cm) | 168±8 | 166±17 | 172±8 | 163±20 |
| Weight (kg) | 70±12 | 62±12 | 66±10 | 60±13 |
| BMI (kg/m2) | 24±3 | 22±2 | 22±3 | 22±2 |
| BSA (m2) | 1.8±0.2 | 1.7±0.3 | 1.8±0.2 | 1.6±0.3 |
| VO2PEAK (% predicted) | 106±23 | 78±18* | 73±14 | 80±20 |
| Exercise (hr/wk) | 5.5±4.5 | 6.8±4.5 | 7.2±5.3 | 6.6±4.4 |
| Medications n (%) | ||||
| Bronchodilators (albuterol, fluticasone/salmeterol) | 0 | 13 (93) | 5 (100) | 8 (89) |
| Pancreatic enzymes | 0 | 12 (86) | 5 (100) | 7 (78) |
| Dornase alfa | 0 | 11 (79) | 4 (80) | 7 (78) |
| Inhaled corticosteroids (fluticasone) | 0 | 1 (7) | 1 (20) | 0 |
| Hypertonic saline | 0 | 1 (7) | 0 | 1 (11) |
| Inhaled antibotics (tobramycin, aztre onam) | 0 | 9 (64) | 5 (100) | 4 (44) |
| Oral antibotics (sulfamethoxazole/trimethoprim azithromycin) | 0 | 5 (36) | 2 (40) | 1 (11) |
| Oral contraceptive | 3 (18) | 0 | 0 | 0 |
BMI = body mass index; BSA = body surface area; VO2peak = maximal oxygen consumption. Data are presented as mean±SD or No. (%)
*
p<0.025 vs. healthy. Statistics not performed on ΔF508 groups.