Table 1– Early characteristics and associations with case status (long-term survival)
AllCasesControlsOR (95% CI)Wald test p-values
Subjects n23078152
Sex
 Female103 (44.8)34 (43.4)69 (45.4)1.00
 Male127 (55.2)44 (56.4)83 (54.6)1.09 (0.62–1.92)0.75
Original presenting features
 Respiratory
  No124 (55.9)53 (68.8)71 (49.0)1.00
  Yes98 (44.1)24 (31.2)74 (51.0)0.44 (0.24–0.80)0.01
 Malabsorption/failure to thrive
  No78 (35.3)33 (42.9)45 (31.3)1.00
  Yes143 (64.7)44 (57.1)99 (68.8)0.59 (0.33–1.06)0.08
 Relative with CF
  No189 (85.9)63 (81.8)126 (88.1)1.00
  Yes31 (14.1)14 (18.2)17 (11.9)1.58 (0.72–3.48)0.26
 Meconium ileus
  No206 (93.6)72 (93.5)134 (93.7)1.00
  Yes14 (6.4)5 (6.5)9 (6.3)1.40 (0.42–4.61)0.58
 Other
  No197 (90.0)67 (87.0)130 (91.6)1.00
  Yes22 (10.1)10 (13.0)12 (8.5)1.67 (0.67–4.17)0.27
 Age at diagnosis yrs
  <5185 (80.4)55 (70.5)130 (85.5)1.000.02
  ≥545 (19.6)23 (29.5)22 (14.5)2.31 (1.17–4.56)
Prior to attendance at adult clinic
 Use of pancreatic enzymes
  No6 (2.6)2 (2.6)4 (2.6)1.000.81
  Yes224 (97.4)76 (97.4)148 (97.4)0.81 (0.14–4.64)
 CFRD before age 16 yrs
  No172 (97.7)66 (97.1)106 (98.2)1.000.64
  Yes4 (2.3)2 (2.9)2 (1.9)1.62 (0.22–12.09)
 Any pneumothorax
  No189 (82.2)77 (98.7)112 (73.7)1.00#<0.01
  Yes41 (17.8)1 (1.3)40 (26.3)0.02 (0.004–0.08)
 Major haemoptysis >100 mL
  No213 (92.6)72 (92.3)141 (92.8)1.00#0.30
  Yes17 (7.4)6 (7.7)11 (7.2)0.58 (0.21–1.63)
At presentation to adult clinic
 Height cm165 (158–172)169 (162–175)162 (157.5–168.5)1.71 (1.04–2.82) per 10 cm0.03
 Weight kg49.5 (43.8–56.2)55.5 (48.8–62.4)45.8 (39.6–50.3)2.25 (1.51–3.34) per 5 kg<0.01
 Body mass index kg·m−217.8 (16.5–19.9)19.6 (17.8–21.5)16.8 (15.7–18.2)1.76 (1.40–2.22)<0.01
 FEV1 % pred41.3 (24.5–69.7)57.3 (41.5–81.6)27.5 (20.4–42.1)1.54 (1.32–1.80) per 5% predicted value<0.01
 FVC % pred61.3 (40.3–81.4)75.9 (60.1–94.8)43.2 (32.3–66.3)1.54 (1.33–1.78) per 5% predicted value<0.01
  • Data are presented as n (%) or median (interquartile range), unless otherwise stated. ORs were derived from weighted logistic regression models. Analysis is limited to those attending from age 16 yrs. ORs were derived from weighted logistic regression models. CF: cystic fibrosis; CFRD: CF-related diabetes mellitus; FEV1: forced expiratory volume in 1 s; % pred: % predicted; FVC: forced vital capacity. #: OR adjusted for age at initial assessment; : OR adjusted for age at initial assessment and sex.