Table 1 Characteristics of the patients at the entry date and associations with case status (death)
All | Death | Survival | Transplant | HR (95% CI) | p-values | |
|---|---|---|---|---|---|---|
Subjects (n) | 39 | 20 (51.3) | 3 (7.7) | 16 (41.0) | ||
Sex, n (%) | 0.84 (0.30–2.35) | 0.743 | ||||
Female | 12 (30.8) | 5 (25.0) | 0 (0.0) | 7 (43.8) | ||
Male | 27 (69.2) | 15 (75.0) | 3 (100.0) | 9 (56.3) | ||
Age (years) | 25.3 ± 8.8 | 23.6 ± 8.7 | 41.7 ± 5.5 | 24.4 ± 6.1 | 0.98 (0.92–1.04) | 0.456 |
Ethnicity – Caucasian (yes) | 37 (94.9) | 19 (51.4) | 3 (8.1) | 15 (40.5) | 0.19 (0.02–1.63) | 0.129 |
Age at diagnosis (years) | 7.0 (11.8) | 4.8 (7.8) | 13.0 (12.5) | 7 (16.2) | 0.98 (0.93–1.04) | 0.560 |
Educational level, n (%) | ||||||
≤ 8 years | 7 (17.9) | 6 (30.0) | 0 (0) | 1 (6.3) | – | 0.639 |
> 8 years and < higher education | 21 (53.8) | 11 (55.0) | 1 (33.3) | 9 (56.3) | 1.34 (0.32–5.6) | 0.685 |
≥ higher education | 11 (28.2) | 3 (15.0) | 2 (66.7) | 6 (37.5) | 0.81 (0.21–3.08) | 0.760 |
Income level monthly, n (%) | ||||||
≤ US$ 231 (≤ 3 BMW) | 16 (41.0) | 11 (55.0) | 0 (0) | 5 (31.3) | – | 0.270 |
> US$ 231 to US$ 693 (> 3 to 10 BMW) | 12 (30.8) | 4 (20.0) | 2 (66.7) | 6 (37.5) | 1.45 (0.50–1.45) | 0.500 |
> US$ 693 (> 10 BMW) | 11 (28.2) | 3 (25.0) | 1 (33.3) | 5 (31.3) | 0.42 (0.08–2.28) | 0.313 |
BMI (kg/m2), mean ± SD | 18.6 ± 2.7 | 17.5 ± 2.2 | 22.6 ± 0.5 | 19.3 ± 2.6 | 0.78 (0.64–0.96) | 0.017 |
F508del mutation | ||||||
Homozygous (yes) | 16 (41.0) | 9 (45.0) | 2 (66.7) | 5 (31.3) | 1.10 (0.44–2.72) | 0.843 |
Heterozygous (yes) | 12 (30.8) | 6 (30.0) | 1 (33.3) | 5 (31.3) | 1.07 (0.41–2.81) | 0.886 |
Pancreatic insufficiency (yes) | 35 (89.7) | 20 (100.0) | 3 (100.0) | 12 (75.0) | 0.04 (0.00–11.4) | 0.261 |
CFRD (yes) | 16 (41.0) | 9 (45.0) | 1 (33.3) | 6 (37.5) | 0.78 (0.31–1.94) | 0.590 |
Any pneumothorax (yes) | 8 (20.5) | 6 (30.0) | 0 (0.0) | 2 12.5) | 0.48 (0.18–1.29) | 0.145 |
Major haemoptysis > 100 mL (yes) | 12 (30.8) | 6 (30.0) | 1 (33.3) | 5 (31.3) | 1.44 (0.52–3.99) | 0.489 |
Bronchial artery embolization (yes) | 8 (20.5) | 3 (15.0) | 1 (33.3) | 4 (25.0) | 2.37 (0.55–10.26) | 0.250 |
CF liver disease (yes) | 18 (46.2) | 12 (60.0) | 1 (33.3) | 5 (31,3) | 0.53 (0.22–1.30) | 0.164 |
Liver transplant (yes) | 1 (2.6) | 1 (5.0) | 0 (0.0) | 0 (0.0) | 0.29 (0.04–2.30) | 0.241 |
ABPA (yes) | 8 (20.5) | 3 (15.0) | 0 (0.0) | 5 (31.3) | 1.94 (0.56–6.70) | 0.296 |
DIOS (yes) | 2 (5.1) | 1 (5.0) | 0 (0.0) | 1 (6.3) | 1.82 (0.24–13.85) | 0.561 |
P. aeruginosa (yes) | 35 (89.7) | 19 (95.0) | 3 (100.0) | 13 (81.3) | 0.29 (0.04–2.21) | 0.234 |
S. aureus (yes) | 30 (76.9) | 17 (85.0) | 2 (66.7) | 11 (68.8) | 0.52 (0.15–1.78) | 0.295 |
MRSA (yes) | 19 (48.7) | 13 (65.0) | 0 (0.0) | 6 (37.5) | 0.54 (0.21–1.35) | 0.185 |
B. cepacia (yes) | 18 (46.2) | 10 (50.0) | 0 (0.0) | 8 (50.0) | 1.13 (0.45–2.83) | 0.799 |
NTM (yes) | 1 (2.6) | 0 (0.0) | 0 (0.0) | 1 (6.3) | ||
Entry date in the study | 1.11 (0.44–2.81) | 0.827 | ||||
≤ 2007 | 11 (28.2) | 7 (35.0) | 0 (0.0) | 4 (25.0) | ||
> 2007 | 28 (71.8) | 13 (65.0) | 3 (100.0) | 12 (75.0) | ||