Table 1.
Controls | Patients with CF | All | ||
---|---|---|---|---|
Lung tissue series | Subjects, n | 38 | 36 | 74 |
Sex, F/M | 21/17 | 20/16 | 41/33 | |
Age, years | 58 ± 14 | 31 ± 9 | 45 ± 18 | |
Genotype, F508del homozygous/F508del heterozygous/other mutations | NA | 22/10/4 | NA | |
FEV1, % predicted (n = 68) | 94 ± 18 | 24 ± 8 | 60 ± 38 | |
Sputum and serum series | Subjects, n | 51 | 67 | 118 |
Sex, F/M | 35/16 | 36/31 | 71/47 | |
Age, years | 31 ± 10 | 34 ± 11 | 33 ± 11 | |
BMI, kg/m² | 23 ± 5 | 22 ± 3 | 22 ± 4 | |
Genotype, F508del homozygous/F508del heterozygous/other mutations | NA | 36/24/7 | NA | |
FEV1, % predicted | 100 ± 9 | 67 ± 23 | 81 ± 25 | |
VC, % predicted | 102 ± 10 | 87 ± 19 | 93 ± 17 | |
Pa infection status, C/I/F/N [3] | NA | 33/12/12/10 | NA | |
Human-derived epithelial cells series | Subjects, n | 7 | 18 | 25 |
Sex, F/M | 1/6 | 9/9 | 10/15 | |
Age, years | 62 ± 8 | 35 ± 12 | 43 ± 16 | |
Genotype, F508del homozygous/F508del heterozygous/other mutations | NA | 9/7/2 | NA | |
FEV1, % predicted (n = 16) | 97 ± 14 | 31 ± 20 | 48 ± 34 | |
Subjects, n (pIgR expression) | 6 | 8 | 14 | |
Subjects, n (UPR activation) | 8 | 10 | 18 | |
Subjects, n (Pa supernatant stimulation) | 0 | 5 | 5 | |
Subjects, n (IL-17 stimulation) | 0 | 6 | 6 |
Definition of abbreviations: CF, cystic fibrosis; F, female; M, male; FEV1, forced expiratory volume in the first second; NA, not applicable; BMI, body mass index; VC, vital capacity; Pa, Pseudomonas aeruginosa; C, chronically infected by Pa; I, intermittently infected by Pa; F, free of Pa infection; N = never infected by Pa; pIgR, polymeric immunoglobulin receptor; UPR, unfolded protein response. N is specified when data are missing.