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

BMI body mass index, FEV1pp percent predicted forced expiratory volume in one second.

1 Adult individuals (≥ 18 years) were classified into BMI categories based on World Health Organization guidelines as underweight (< 18.5 kg/m²), adequate weight (18.5–24.9 kg/m²), or overweight (≥ 25.0 kg/m²). Children were classified as underweight (< 10th percentile), adequate weight (10-85th percentile), or overweight (> 85th percentile). * p < 0.05. **p < 0.001.