Table 2. General characteristics of cystic fibrosis (CF) patients according to 30-year mortality.
| Characteristics | Death <30 years(n=8) | Survivors(n=40) | P |
|---|---|---|---|
| Male, n (%) | 6 (75) | 22 (55) | 0.440 |
| Initial presentation of the disease | |||
| Diagnostic age (years), median (IQR) | 1.5 (6.25) | 2.5 (9.75) | 0.559 |
| Respiratory symptoms, n (%) | 7 (87.5) | 35 (87.5) | 1.0 |
| Steatorrhea, n (%) | 7 (87.5) | 23 (57.5) | 0.229 |
| Meconium ileus, n (%) | 0 (0) | 3 (7.5) | 1.0 |
| F508del homozygosity, n (%) | 4 (50) | 6 (15) | 0.028* |
| CF-related complications before age 18 | |||
| Pancreatic insufficiency, n (%) | 7 (87.5) | 32 (80) | 1.0 |
| CFRDM, n (%) | 1 (12.5) | 2 (5) | 0.429 |
| Use of ursodeoxycholic acid, n (%) | 4 (50) | 15 (37.5) | 0.695 |
| Massive hemoptysis, n (%) | 3 (37.5) | 2 (5) | 0.027* |
| MSSA, n (%) | 4 (50) | 25 (62.5) | 0.695 |
| MRSA, n (%) | 2 (25) | 7 (17.5) | 0.633 |
| Pseudomonas aeruginosa, n (%) | 8 (100) | 33 (82.5) | 0.583 |
| Burkholderia cepacia, n (%) | 3 (37.5) | 10 (25) | 0.664 |
| ABPA, n (%) | 2 (25) | 1 (2.5) | 0.068 |
| Intravenous azithromycin, n (%) | 7 (87.5) | 24 (60) | 0.230 |
| Inhaled colistin, n (%) | 4 (50) | 16 (40) | 0.703 |
| Inhaled tobramycin, n (%) | 4 (50) | 12 (30) | 0.413 |
| Hospital admission, n (%) | 8 (100) | 32 (80) | 0.320 |
| ICU admission, n (%) | 1 (12.5) | 3 (7.5) | 0.530 |
| Invasive mechanical ventilation, n (%) | 2 (25) | 3 (7.5) | 0.189 |
| Clinical data at age 18 years | |||
| Body mass index (kg/m2), mean (SD) | 19.99 (2.20) | 20.42 (2.37) | 0.640 |
| FEV1 (L), mean (SD) | 1.85 (0.54) | 2.73 (0.88) | 0.01* |
| FEV1 (%), mean (SD) | 42.5 (10.73) | 68.7 (21.3) | 0.002* |
| FVC (L), mean (SD) | 2.87 (0.81) | 3.51 (0.95) | 0.088 |
| FVC (%), mean (SD) | 58.87 (12.35) | 77 (18.15) | 0.01* |
| FEV1/FVC (absolute), mean (SD) | 64.6 (6.4) | 77.3 (9.81) | 0.001* |
| FEV1/FVC (%), mean (SD) | 74.75 (7.59) | 89.64 (11.69) | 0.001* |
Data are reported as n (%), median (interquartile range), or mean (SD). CF: cystic fibrosis; CFRDM: CF related diabetes mellitus; MSSA: methicillin-sensitive Staphylococcus aureus; MRSA: methicillin-resistant Staphylococcus aureus; ABPA: allergic bronchopulmonary aspergillosis; ICU: intensive care unit; FEV1: first second of forced expiratory volume; FVC: forced vital capacity; L: value in liters. *P<0.05.