Table 1.
Characteristics of patients included in the study (N = 122)1
| Male | 48.8 % |
|---|---|
| Age |
246.68 ± 168,73 months (87 - 932 months) |
| Caucasoid |
93.4% |
| BMI - Thinness and Thinness accentuated |
22.3% |
| SaO2 |
94.87 ± 4.53 (66 – 99) |
| Bhalla |
9.41 ± 5.57 (0 – 25) |
| Kanga |
19.37 ± 5.01 (11 – 40) |
| Shwachman-Kulczycki |
65.41 ± 16.02 (20 – 95) |
| FVC (%) |
78.27 ± 22.86 (19 – 135) |
| FEV1 (%) |
70.28 ± 26.17 (17 – 125) |
| FEV1/FVC (%) |
83.83 ± 15.79 (37 – 137) |
| FEF25-75% |
58.50 ± 34.83 (7 – 150) |
| FVC (%) reversibility |
0.92 ± 10.48 (-27 – 32) |
| FEV1 (%) reversibility |
2.15 ± 9.45 (-12 – 31) |
| FEV1/FVC (%) reversibility |
2.84 ± 9.69 (-19 – 47) |
| FEF25-75% reversibility |
7.24 ± 9.43(-12 – 30) |
| Nasal Polyps |
21.7% |
| Diabetes mellitus |
20.8% |
| Osteoporosis |
20.8% |
| Pancreatic insufficiency |
76% |
| Meconium ileus |
9.1% |
|
P. aeruginosa status 2 |
53.7% |
|
P. aeruginosa mucoid status 2 |
45.5% |
|
B. cepacia status 2 |
9.1% |
|
A. xylosoxidans status 2 |
9.9% |
|
S. aureus status 2 |
78.5% |
|
CFTR mutation |
|
| F508del/F508del |
29 (24%) |
| F508del/G542X |
10 (8.3%) |
| F508del/N1303K |
3 (2.5%) |
| F508del/R1162X |
3 (2.5%) |
| F508del/R553X |
1 (0.8%) |
| G542X/I618T |
1 (0.8%) |
| G542X/R1162X |
1 (0.8%) |
| F508del/No identified mutation |
26 (21.5%) |
| G542X/No identified mutation |
4 (3.3%) |
| No identified mutation | 43 (35.3%) |
N - Sample size; BMI - body mass index; % - percentage; FVC - forced vital capacity; FEV1 - forced expiratory volume in the first second; FEF25-75% - forced expiratory flow between 25 and 75% of CVF. 1. Continuous variables expressed as mean ± SD (range). 2. Based on 3 consecutive positive respiratory cultures.