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

Table 2 Pulmonary function tests, 6-min walk distance and pulmonary artery systolic pressure estimated by echocardiography 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)
FVC (% predicted)	40.5 ± 10.3	42.6 ± 12.9	35.7 ± 4.1	38.9 ± 8.4	1.00 (0.96–1.05)	0.837
FEV₁(% predicted)	24.4 ± 3.9	24.3 ± 4.2	23.3 ± 4.6	24.8 ± 3.7	0.95 (0.84–1.07)	0.362
FEV₁/FVC (%)	54.7 ± 9.4	53.6 ± 10.5	55.4 ± 3.5	55.9 ± 8.9	0.98 (0.93–1.04)	0.507
SpO₂(%)	91.6 ± 4.3	92.7 ± 3.3	87.3 ± 4.5	91.2 ± 5.1	1.03 (0.91–1.2)	0.629
6-MWT (m)	479.9 ± 104.8	481.7 ± 115.3	514.0 ± 51.9	471.6 ± 103.9	0.99 (0.99–1.00)	0.840
PASP ≥ 35 mmHg (yes)	15 (38.5)	6 (30.0)	1 (33.3)	8 (50.0)	2.17 (0.83–5.72)	0.116

Data are presented as n (%) or median ± standard deviation. Hazard ratios (HRs) were derived from Cox regression models. CI confidence interval, FVC forced vital capacity, FEV₁ forced expiratory volume in the first second, SpO₂ room air saturation of peripheral oxygen (evaluated by a non-invasive pulse oximeter), 6-MWd 6-min walk distance, PASP pulmonary artery systolic pressure

ISSN: 1471-2466