CF-PI + CF-PS | CF-PI | CF-PS | CFTR dysfunction | CF unlikely | CF vs CFTR dysfunction | CF-PI vs CF-PS | CF-PS vs CFTR dysfunction | CF-PS vs CF unlikely | CFTR dysfunction vs CF unlikely | |
Subjects (n) | 165 | 62 | 103 | 59 | 46 | NS | NS | NS | NS | NS |
Current age (years)† | 19.7 (13.4–29.5) | 19.5 (10.5–25.7) | 19.8 (13.5–34.5) | 19.3 (13.7–34.0) | 16.6 (9.5–28.5) | |||||
Gender (M/F) | 89/76 | 39/23 | 50/53 | 26/33 | 24/22 | 0.19 | 0.07 | 0.58 | 0.68 | 0.41 |
Age at time of diagnosis (years)† | 3.0 (0.3–10.7) | 0.2 (0.1–0.9) | 7.4 (2.7–19.6) | 11.3 (5.8–24.6) | 13.9 (4.9–24.5) | <0.001 | <0.001 | 0.03 | 0.01 | 0.60 |
Sweat chloride (mmol/l)† | 96.0 (78.0–103.0) | 102.0 (96.8–110.0) | 85.0 (73.0–98.9) | 45.7 (38.8–53.9) | 40.3 (36.0–50.1) | <0.001 | <0.001 | <0.001 | <0.001 | 0.05 |
Z-scores* | ||||||||||
For weight | −0.08 (1.25) | −0.40 (1.04) | 0.10 (1.33) | 0.09 (0.98) | −0.53 (1.65) | 0.64 | 0.05 | 0.99 | 0.14 | 0.16 |
For height | −0.09 (1.03) | −0.12 (0.97) | −0.07 (1.06) | 0.13 (1.03) | −0.26 (1.32) | NS | NS | NS | NS | NS |
For BMI | −0.16 (1.30) | −0.46 (1.16) | 0.02 (1.36) | −0.08 (1.03) | −0.55 (1.80) | 0.95 | 0.09 | 0.99 | 0.29 | 0.51 |
Most recent FEV1% predicted† | 78 (60–94) | 73 (52–87) | 83 (62–97) | 89 (64–105) | 91 (75–106) | 0.02 | 0.02 | 0.15 | 0.03 | 0.66 |
Recurrent LRTI‡ | 83 | 100 | 71 | 71 | 44 | 0.06 | <0.001 | 0.99 | 0.005 | 0.01 |
Sputa‡ | 66 | 86 | 54 | 48 | 42 | 0.02 | 0.001 | 0.46 | 0.25 | 0.60 |
P aeruginosa colonisation‡ | 39 | 53 | 30 | 17 | 5 | 0.002 | 0.004 | 0.06 | <0.001 | 0.07 |
S aureus or P aeruginosa isolation‡ | 86 | 100 | 78 | 65 | 14 | <0.001 | <0.001 | 0.05 | <0.001 | <0.001 |
Clubbing‡ | 51 | 71 | 36 | 27 | 2 | 0.003 | <0.001 | 0.26 | <0.001 | 0.001 |
ABPA‡ | 15 | 21 | 11 | 10 | 2 | 0.38 | 0.08 | 0.89 | 0.11 | 0.14 |
Nasal polyposis‡ | 44 | 82 | 21 | 14 | 18 | <0.001 | <0.001 | 0.22 | 0.66 | 0.56 |
Pancreatitis‡ | 9 | 2 | 14 | 14 | 7 | 0.31 | 0.01 | 0.97 | 0.27 | 0.34 |
MI/DIOS‡ | 21 | 42 | 8 | 0 | 0 | <0.001 | <0.001 | 0.05 | 0.11 | 0.99 |
Liver disease/PTH/CL‡ | 15 | 19 | 13 | 2 | 0 | 0.007 | 0.24 | 0.04 | 0.04 | 0.99 |
Data are presented as *mean (SD), †median (interquartile range) or ‡percentage unless otherwise stated.
CF-PI, sweat chloride concentration >60 mmol/l and pancreatic insufficiency; CF-PS, sweat chloride concentration >60 mmol/l and pancreatic sufficiency; CFTR dysfunction, sweat chloride concentration 30–60 mmol/l and two mutations identified and/or abnormal nasal potential difference (NPD); CF unlikely, patients with an intermediate sweat chloride concentration but no detection of two CFTR mutations and/or normal NPD.
ABPA, allergic bronchopulmonary aspergillosis; CL, choledocolithiasis; DIOS, distal intestinal obstruction syndrome; FEV1, forced expiratory volume in 1 s; LRTI, lower respiratory tract infection; MI, meconium ileus; PTH, portal hypertension; P aeruginosa, Pseudomonas aeruginosa; S aureus, Staphylococcus aureus.
p Values ⩽0.05 are shown in bold type. The H-values for age at diagnosis, sweat chloride value and for FEV1% predicted were all significant at the level of 0.001. However, the F-value for height (ANOVA) and the H-value for current age (Kruskal-Wallis) were not significant.