G85E | PS | PI | G85E versus PS | |
Subjects n | 55 | 44 | 55 | |
Current age yrs | 18.2±9 (2–51) | 18.2±9 (2–52) | 18.9±9 (3–46) | 0.06# |
Age at time of diagnosis months | 22 (3–124) | 44 (6–169) | 6 (2–28) | 0.13¶ |
Sweat chloride mEq·L−1 | 106 (95–115) | 100 (78–109) | 102 (94–110) | 0.0018¶ |
Presenting symptoms | ||||
Steatorrhea | 40 | 5 | 84 | 0.0001+ |
Failure to thrive | 40 | 14 | 71 | 0.0001+ |
Meconium ileus | 2 | 0 | 12 | 0.49+ |
Respiratory | 78 | 74 | 63 | 0.17+ |
Pancreatic insufficiency | 54 | 0 | 100 | <0.0001+ |
Most recent weight for height percentile | 57±4 | 72±5 | 58±5 | 0.02# |
Most recent FEV1 % pred | 69±5 | 85±7 | 66±5 | 0.08# |
Chronic P. aeruginosa colonisation | 42 | 23 | 46 | 0.0083+ |
Complications | ||||
Liver cirrhosis | 9 | 0 | 11 | <0.05+ |
CF‐RD | 11 | 2 | 13 | 0.1+ |
DIOS | 15 | 7 | 9 | 0.11+ |
Pancreatitis n | 2 | 2 | 0 | 0.55+ |
Data are presented as mean±sd (range), median (interquartile range), mean±sem or % unless otherwise stated
Subjects were matched for centre, sex and age
FEV1: forced expiratory volume in one second; pred: predicted
P. aeruginosa: Pseudomonas aeruginosa
CF‐RD: cystic fibrosis-related diabetes
DIOS: distal intestinal obstruction syndrome
Statistical analysis by paired t‐test#, Wilcoxon signed-ranks test¶ and Fisher exact test+