Table 1 Characteristics of children with cystic fibrosis and healthy controls.
| CFA (n = 16) | CFB (n = 24) | Healthy (n = 12) | |
|---|---|---|---|
| Age (years)* | 1.15 (0.83) | 2.7 (1.5) | 3.3 (1.2) |
| M/F | 9/7 | 11/13 | 8/4 |
| Symptom score‡ | 13.5 (2.2) | 14.6 (2.7) | NA |
| Cells from TB (×106)* | 1.84 (0.67) | 1.9 (1.07) | 1.52 (0.7) |
| Genotype | |||
| dF508 homozygote | 50% | 66.7% | NA |
| dF508 heteroygote¶ | 43.75% | 29.2% | |
| G542X/G542X | – | 4.2% | |
| Others§ | 6.25% | – | |
| Cells in BAL fluid | |||
| TCC (cells×103/ml)† | 345.3 (236 to 506) | 443.5 (285 to 690) | NA |
| Neutrophils (cells ×103/ml)† | 55.9 (29.5 to 105.9) | 88.4 (43.2 to 181.1) | |
| Neutrophils (%)* | 22.6 (18.9) | 28.3 (23.2) | |
| iNOS expression† | 0.23 (0.11 to 0.46) | 0.22 (0.14 to 0.34) | 0.76 (0.51 to 1.14) |
SD, standard deviation; BAL, bronchoalveolar lavage; TB, tracheobronchial brushing; TCC, total cell count per ml BAL fluid; CFA, CF without bacterial infection at actual or previous BAL; CFB, CF with bacterial infection either in present or previous BAL.
*Mean (SD).
†Geometric mean (95% confidence interval).
‡Cystic fibrosis clinical score.15
¶ Heterozygote mutations included: unknown mutation (n = 5), N310, G551D, R117(H, R553X, P67L, S1235R, W1282 (n = 1).
§This patient was compound heterozygote with one unidentified allele and a positive sweat test.