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Table 1 Characteristics of the patients at the entry date and associations with case status (death)

From: Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil

  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)   
  1. Data are presented as n (%), median ± standard deviation or median (interquartile range). Hazard ratios (HRs) were derived from Cox regression models. CI confidence interval, BMW Brazilian minimum wage, BMI body mass index, CFRD cystic fibrosis related diabetes, ABPA allergic bronchopulmonary aspergillosis, DIOS distal Intestinal obstruction syndrome, MRSA methicillin-resistant Staphylococcus aureus, NTM non-tuberculous mycobacteria