TABLE 1

Overall survival of children with cystic fibrosis diagnosed by neonatal screening or symptoms or with meconium ileus

PatientsEvents#20-year survival % (95% CI)30-year survival % (95% CI)p-value
NBS342 (100)3592.35 (88.03–95.16)80.11 (71.42–86.40)
 Sex
  Male167 (49)1591.86 (85.26–95.58)84.95 (74.51–91.35)0.3
  Female175 (51)2092.90 (86.14–96.43)73.70 (57.27–84.61)
 Pancreatic status at birth+
  Sufficient60 (18)196.15 (75.69–99.45)96.15 (75.69–99.45)0.10
  Insufficient281 (82)3491.75 (86.96–94.84)78.34 (68.99–85.18)
 Birth cohort
  1971–198030 (9)1074.90 (54.33–87.20)67.20 (46.26–81.47)0.2
  1981–199092 (27)1893.34 (85.78–96.95)79.89 (67.80–87.83)
  1991–2000119 (35)696.40 (90.68–98.63)
  2001–2014101 (29)1
Symptoms143 (100)5082.98 (75.49–88.35)71.03 (62.21–78.15)
 Sex
  Male74 (52)2776.93 (65.10–85.19)69.58 (56.69–79.32)0.43
  Female69 (48)2389.29 (78.83–94.75)72.82 (59.89–82.19)
 Pancreatic status at birth+
  Sufficient48 (34)00.000.00<0.001
  Insufficient94 (66)4975.40 (65.07–83.06)58.50 (47.32–68.10)
 Birth cohort
  1971–198096 (67)4677.08 (67.32–84.27)65.22 (54.70–73.87)0.06
  1981–199031 (22)496.55 (77.95–99.51)84.69 (63.79–94.05)
  1991–200010 (7)0100.00
  2001–20116 (4)0
Meconium ileus101 (100)1890.64 (82.04–95.24)71.77 (56.47–82.49)

Data are presented as n (%) or n, unless otherwise stated. NBS: newborn screening. #: death due to any cause is considered an event; : log-rank test; +: data were missing in one case.