Table 7.
Patient characteristics (n = 180)
| Characteristic | |
|---|---|
| Male gender |
50% (90) |
| Age (months) |
212 ± 15.75 (7–288) |
| Caucasoid |
91.75% |
| BMI - thinness and accentuated thinness |
22.22% (40) |
| One class I, II or III identified mutation |
28.33% (51) |
| Two class I, II or III identified mutations |
47.22% (85) |
| Age at first clinical manifestation (months) |
35 ± 8.88 (0–156) |
| Age at diagnosis (months) |
87 ± 13.63 (0–170.76) |
| Age at start of digestive symptoms (months) |
40.6 ± 9.11 (0–149.4) |
| Age at start of pulmonary symptoms (months) |
34.8 ± 9.88 (0–1156) |
| SpO2(%) |
94.92 ± 4.26 (66–99) |
| Bhalla |
8.74 ± 5.72 (0–25) |
| Kanga |
18.85 ± 5.84 (10–40) |
| Shwachman-Kulczycki |
65.85 ± 16.77 (20–95) |
| FVC(%) |
79.29 ± 23.55 (19–135) |
| FEV1(%) |
71.29 ± 27.467 (17–132) |
| FEV1/FVC(%) |
83.46 ± 15.95 (37–137) |
| FEF25–75% |
59.05 ± 35.55 (7–150) |
| Nasal polyps |
18.33% (33) |
| Diabetes mellitus |
18.33% (33) |
| Osteoporosis |
16.11% (29) |
| Pancreatic insufficiency |
80.0% (144) |
| Meconium ileus |
15.00% (27) |
| Age at first isolated P. aeruginosa (months) |
102.6 ± 14.45 (24–180) |
|
P. aeruginosa statusa |
56.67% (102) |
|
P. aeruginosa mucoid statusa |
42.22% (76) |
|
B. cepacia statusa |
13.88% (25) |
|
A. xylosoxidans statusa |
10.00% (18) |
| S. aureus statusa | 78.88% (142) |
Continuous variables expressed as mean ± SD (range). Other data shown as percentage (number of patients). aBased on three consecutive positive respiratory cultures.
N, Sample size; BMI, Body mass index; SpO2, Hemoglobin oxygen saturation in the blood; FVC, Forced vital capacity; FEV1, Forced expiratory volume in the first second; FEF25–75, Forced expiratory flow between 25 and 75% of FVC.