Table 2 Univariate and multivariable Cox proportional hazard models for death adjusted for demographic and clinical characteristics (2005–2020).
Univariate model | Multivariable model | |||
|---|---|---|---|---|
Hazard ratio | 95% CI | Hazard ratio | 95% CI | |
Sex | ||||
Female | 1.00 | – | 1.00 | – |
Male | 0.91 | 0.77 – 1.09 | 1.00 | 0.83 – 1.19 |
Diagnosis category | ||||
Diagnosed with meconium ileus | 1.00 | – | 1.00 | – |
Age < 6 weeks or through NBS/Prenatal screening | 1.06 | 0.78 – 1.45 | 1.37 | 1.00 – 1.88* |
Age 6 weeks– < 2 years | 1.12 | 0.79 – 1.59 | 1.12 | 0.79 – 1.59 |
Age 2 – 17 years | 0.74 | 0.49 – 1.12 | 0.85 | 0.56 – 1.29 |
Age ≥ 18 years | 0.60 | 0.37 – 1.00* | 0.88 | 0.52 – 1.50 |
F508del homozygous | ||||
No | 1.00 | – | 1.00 | – |
Yes | 1.12 | 0.93 – 1.33 | 1.09 | 0.91 – 1.30 |
Lung transplant | ||||
No | 1.00 | – | 1.00 | – |
Yes | 9.49 | 7.83 – 11.51** | 3.56 | 2.84 – 4.47** |
Pancreatic exocrine status | ||||
Sufficient | 1.00 | – | 1.00 | – |
Insufficient | 1.33 | 1.07 – 1.64* | 1.13 | 0.91 – 1.40 |
Missing | 1.17 | 0.80 – 1.69 | 1.14 | 0.78 – 1.66 |
FEV1 pp | ||||
≥ 70 | 1.00 | – | 1.00 | – |
40–69 | 5.49 | 3.98 – 7.56** | 4.60 | 3.33 – 6.36** |
< 40 | 22.32 | 16.2 – 30.75** | 9.77 | 6.84 – 13.96** |
Missing | 3.71 | 2.3 – 5.98** | 4.95 | 1.76 – 13.88* |
BMI1 | ||||
Adequate weight status | 1.00 | – | 1.00 | – |
Underweight | 4.42 | 3.6 – 5.42** | 1.93 | 1.54– 2.41** |
Overweight | 0.41 | 0.3 – 0.56** | 0.79 | 0.57 – 1.08 |
Missing | 0.7 | 0.46 – 1.08 | 0.61 | 0.21 – 1.77 |
