| CF(n = 43) | PCD(n = 7) | CRS(n = 26) | Controls(n = 7) | |
| Age (years) | 6.2 (0.3–16.8) | 9.2 (5.7–14.8) | 4.8 (0.9–15.2) | 5.8 (0.3–16.3) |
| Sex, male (%) | 14 (33%) | 2 (29%) | 13 (50%) | 4 (57%) |
| FEV1 (% predicted)† | 60 (29–91) | 66 (46–89) | 89 (75–94)*** | 87 (82–118) |
| FVC (% predicted)† | 81 (32–114) | 82 (48–102) | 103 (82–118)** | 97 (86–108) |
| Total cells (×106/ml) | 1.7 (0.21–28.5) | 0.7 (0.16–14.80) | 0.3 (0.12–20.43)*** | 0.3 (0.12–0.46)*** |
| Neutrophils (×106/ml) | 0.7 (0.02–23.97) | 0.3 (0.05–13.76) | 0.0 (0.00–17.91)*** | 0.0 (0.00–0.01)*** |
| Eosinophils (×106/ml) | 0.0 (0.00–0.22) | 0.0 (0.00–0.09) | 0.0 (0.00–0.10)** | 0.0 (0.00–0.00)** |
| IL-8 (pg/ml) | 1340 (31–1760) | 1272 (714–1374) | 703 (31–1560)*** | 64 (31–221)*** |
| IL-10‡ (pg/ml) | 15 (15–74) | 44 (15–68) | 32 (15–108)*** | 29 (24–51)* |
| IL-13‡ (pg/ml) | 23 (23–95) | 23 (23–23) | 27 (23–73)** | 26 (23–82)** |
| TGF-β1‡ (pg/ml) | 78 (2–265) | 131 (22–331) | 17 (2–211)*** | 2 (2–8)*** |
| Neutrophil elastase (μm/ml) | 201 (100–5000) | 180 (100–3502) | 100 (100–1297)*** | 100 (100–100)** |
| MMP-9 (ng/ml) | 185 (2–2454) | 221 (59–3334) | 46 (1–3188)*** | 4 (1–6)*** |
| TIMP-1 (ng/ml) | 105 (7–2629) | 150 (26–770) | 113 (11–994) | 39 (13–214) |
| Molar ratio MMP9:TIMP-1 | 0.4 (0.01–28.16) | 0.4 (0.02–35.05) | 0.0 (0.01–26.51)*** | 0.0 (0.00–0.08)*** |
| Glycosaminoglycans (μg/ml) | 59 (2–120) | 61 (2–89) | 2 (2–48)*** | 2 (2–7)*** |
| Elastin (μg/ml) | 424 (125–2000) | 417 (125–2000) | 149 (125–1698)** | 54 (125–204)** |
| Collagen (μg/ml) | 25 (5–412) | 195 (5–500) | 5 (5–68)*** | 5 (5–5)*** |
CF, cystic fibrosis; PCD, primary ciliary dyskinesia; CRS, chronic respiratory symptoms; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; IL, interleukin; TGF-β1, transforming growth factor-β1; MMP-9, matrix metalloproteinase-9; TIMP-1, tissue inhibitor of metalloproteinases-1.
Values are shown as median (range).
*p<0.017, **p<0.01, ***p<0.001 for CF vs PCD, CRS or controls.
†Number of children performing spirometry: CF, n = 23; PCD, n = 7, CRS, n = 7, controls, n = 2.
‡Measured with protease inhibitors.