| Characteristic |
Total number of patients |
Data distribution |
| Female gender |
169 |
87 (51.5) |
| Age (months) |
168 |
154; 177.13 (95%CI: 157.26 – 197.01) ± 77.71; 87 – 468 |
| Caucasoid |
169 |
158 (93.5) |
| BMI - thinness and accentuated thinness |
167 |
34 (20.4) |
| One class I, II or III identified mutation |
|
39 (23.1) |
| Two class I, II or III identified mutations |
|
90 (53.3) |
| Age at first clinical manifestation (months) |
159 |
2; 4.607 (95%CI: 2.775 – 6.438) ± 7.151; 0 – 39 |
| Age at diagnosis (months) |
162 |
16; 36.197 (95%CI: 21.528 – 50.865) ± 57.27; 0 – 379 |
| Age at start of digestive symptoms (months) |
140 |
3; 18.672 (95%CI: 5.189 – 32.156) ± 52.6465; 0 – 381 |
| Age at start of pulmonary symptoms (months) |
154 |
4; 11 (4.431 – 17.569) ± 25.649; 0 – 187 |
| SpO2 (%) |
162 |
97; 95.164 (93.95 – 96.378) ± 4.741; 66 – 99 |
| Bhalla score |
122 |
8; 8.697 (7.441 – 9.772) ± 4.551; 0 – 23 |
| Kanga score |
131 |
18; 19.361 (17.915 – 20.806) ± 5.645; 11 – 40 |
| Shwachman-Kulczycki score |
144 |
65; 67.049 (63.255 – 70.843) ± 14.815; 20 – 95 |
| FVC (%) |
126 |
82; 82.295 (76.739 – 87.851) ± 21.693; 19 – 132 |
| FEV1 (%) |
124 |
73; 75.131 (68.831 – 81.432) ± 24.601; 19 – 132 |
| FEV1/FVC (%) |
123 |
86; 83.937 (80.617 – 87.317) ± 13079; 37 – 100 |
| FEF25-75% |
124 |
62; 62.066 (54.110 – 70.021) ± 31.062; 8 – 137 |
| Nasal polyps |
166 |
32 (19.3) |
| Diabetes mellitus |
166 |
30 (18.1) |
| Osteoporosis |
166 |
28 (16.9) |
| Pancreatic insufficiency |
168 |
136 (81) |
| Meconium ileus |
168 |
25 (14.9) |
| Age at first isolated P. aeruginosa (months) |
122 |
31; 54.066 (37.578 – 70.553) ± 64.375; 4 – 379 |
| P. aeruginosastatusa |
169 |
91 (53.8) |
| P. aeruginosamucoidstatusa |
169 |
70 (41) |
| B. cepaciastatusa |
169 |
17 (10.1) |
| A. xylosoxidansstatusa |
169 |
23 (13.6) |
| S. aureusstatusa |
169 |
134 (79.3) |
