TABLE 2

Survival analysis of cystic fibrosis (CF) patients according to clinical severity in the first 3 years of life and diagnosis

	Patients	Events	10-year survival % (95% CI)	15-year survival % (95% CI)	20-year survival % (95% CI)	p-value
Overall survival^#
Severe
NBS	95	23	98.84 (92.03–99.84)	92.56 (84.17–96.59)	84.93 (74.29–91.41)	0.007^§
Symptoms	34	21	87.88 (70.86–95.27)	72.73 (54.13–84.77)	63.64 (44.95–77.46)	0.007^§
Moderate
NBS	80	4	100.00	98.46 (89.58–99.78)	94.48 (83.72–98.20)	0.016^§
Symptoms	40	12	100.00	88.89 (73.05–95.68)	85.93 (69.41–93.89)	0.016^§
Mild
NBS	149	6	99.29 (95.07–99.90)	99.29 (95.07–99.90)	98.21 (92.83–99.56)	0.6^§
Symptoms	58	12	96.55 (86.91–99.13)	96.55 (86.91–99.13)	92.80 (81.92–97.24)	0.6^§
Cumulative incidence of CF-related death^¶
Severe
NBS	95	13	1.16 (0.17–8.16)	6.22 (2.66–14.55)	12.36 (6.66–22.92)	0.0001^ƒ
Symptoms	34	19	12.12 (4.84–30.38)	27.27 (15.62–47.61)	36.36 (23.16–57.11)	0.0001^ƒ
Moderate
NBS	80	3	0.00	0.00	3.98 (1.02–15.48)	0.01^ƒ
Symptoms	40	10	0.00	11.11 (4.41–27.99)	14.07 (6.24–31.75)	0.01^ƒ
Mild
NBS	149	4	0.71 (0.10–5.00)	0.71 (0.10–5.00)	1.79 (0.44–7.28)	0.2^ƒ
Symptoms	58	9	3.45 (0.88–13.46)	3.45 (0.88–13.46)	7.20 (2.80–18.53)	0.2^ƒ
Event-free survival⁺
Severe
NBS	95	33	98.84 (92.03–99.84)	88.68 (79.34–93.95)	79.76 (68.53–87.33)	0.07^§
Symptoms	34	25	87.88 (70.86–95.27)	72.73 (54.13–84.77)	63.64 (44.95–77.46)	0.07^§
Moderate
NBS	80	6	100.00	98.46 (89.58–99.78)	92.68 (81.55–97.21)	0.013^§
Symptoms	40	15	100.00	88.89 (73.05–95.68)	85.93 (69.41–93.89)	0.013^§
Mild
NBS	149	8	99.29 (95.07–99.90)	99.29 (95.07–99.90)	95.78 (88.94–98.43)	0.8^§
Symptoms	58	14	96.55 (86.91–99.13)	96.55 (86.91–99.13)	92.80 (81.92–97.24)	0.8^§

Data are presented as n, unless otherwise stated. NBS: newborn screening. ^#: death due to any cause is considered an event; ^¶: CF-related death is considered an event; ⁺: death due to any cause and lung transplant were considered events; ^§: log-rank test; ^ƒ: Gray test.