Table 1

Characteristics of the study populations for each clinical outcome stratified by diagnosis by NBS

WeightBMI%FEV1 cPA
Clin. diag.Diag. by NBSClin. diag.Diag. by NBSClin. diag.Diag. by NBSClin. diag.Diag. by NBS
N1799146817921460144477218271526
Age range in years1–15.91–15.61–15.81–15.95–15.95–15.60–15.90–15.9
Male (%)904 (50.3)757 (51.6)900 (50.2)754 (51.6)712 (49.3)401 (51.9)920 (50.4)787 (51.6)
Non-white ethnicity (%)116 (6.4)53 (3.6)116 (6.5)52 (3.6)87 (6.0)32 (4.1)116 (6.3)57 (3.7)
Born abroad (%)102 (5.7)35 (2.4)101 (5.6)35 (2.4)81 (5.6)27 (3.5)102 (5.6)39 (2.6)
F508 class (%)
 Homozygous1005 (55.9)756 (51.5)1004 (56.0)755 (51.7)821 (56.9)398 (51.6)1016 (55.6)789 (51.7)
 Heterozygous608 (33.8)595 (40.5)603 (33.6)589 (40.3)478 (33.1)305 (39.5)625 (34.2)615 (40.3)
 Other186 (10.3)117 (8.0)185 (10.3)116 (7.9)145 (10.0)69 (8.9)186 (10.2)122 (8.0)
IMD z-score (median (IQR))−0.29 (−0.79- 0.53)−0.29 (−0.79- 0.66)−0.29 (−0.79- 0.53)−0.29 (−0.79- 0.66)−0.29 (−0.79- 0.53)−0.29 (−0.79-0.53)−0.29 (−0.79- 0.53)−0.29 (−0.79- 0.66)
Meconium Ileus (%)596 (33.1)0 (0.0)593 (33.1)0 (0.0)425 (29.4)0 (0.0)611 (33.4)0 (0.0)
Pancreatic insufficient (%)1592 (88.5)1227 (83.6)1589 (88.7)1223 (83.8)1293 (89.5)659 (85.4)1614 (88.3)1276 (83.6)
Born pre-2007 (%)1192 (66.3)289(19.7)1192 (66.5)289 (19.8)1174 (81.3)287 (37.2)1193 (65.3)289 (18.9)
Median age at diagnosis in years (IQR)0.14 (0–7.82)0.07 (0–0.42)0.14 (0–7.84)0.07 (0–0.42)0.24 (0–8.45)0.07 (0–0.5)0.13 (0–7.75)0.07 (0–0.41)
Mean outcome (SD) between the ages 1 and 2 for weight and bmi and ages 5 and 6 for %FEV1 0.07 (1.05)0.26 (1.03)0.58 (1.11)0.7 (1.03)87.88 (16.38)89.22 (15.76)NANA
Mean FVC between ages 5 and 6 (SD)NANANANA93.37 (17.24)94.66 (15.46)NANA
Median FEV1/FVC between ages 5 and 6 (IQR)NANANANA0.96 (0.89–1.02)0.96 (0.89–1)NANA
  • BMI, body mass index; cPA, chronic Pseudomonas aeruginosa; FEV1, forced expiratory volume in one second; FVC, forced vital capacity; NBS, newborn bloodspot screening.