Table 1.
Variable of interest | Healthy cohort | Patient cohort |
---|---|---|
Number of subjects (n) | 52 | 41 |
Number of subjects in longitudinal cohort | 0 | 11 |
Agea | ||
Median age at sample collection in months (age range) | 11 (1–75) | 26 (0–82) |
Median age at diagnosis in months (age range) | Not applicable | 8 (0–39) |
Number of subjects in age groups: 0, 1–3, 4–6 years | 28, 9, 15 | 5, 20, 16 |
Genderb | ||
Number of female subjects (in %) | 21 (40%) | 14 (34%) |
Number of male subjects (in %) | 31 (60%) | 27 (66%) |
Number of samples collected at different locations | ||
Kindergarten | 17 (33%) | 0 |
Local paediatrician (preventive medical check-up) | 16 (31%) | 0 |
Parent–child groups | 19 (36%) | 0 |
CF outpatient clinic | 0 | 41 (100%) |
First clinical indication for CF | ||
CF newborn screening | Not applicable | 15 (37%)c |
Family history | Not applicable | 4 (10%) |
Meconium ileus | Not applicable | 4 (10%) |
Gastrointestinal and/or pulmonary symptoms | Not applicable | 18 (43%) |
Pancreatic state | ||
Pancreatic insufficient (PI) | 0 | 33 (80%) |
Pancreatic sufficient (PS) | 52 (100%) | 8 (20%) |
Class of CFTR mutation | ||
II/II | Not applicable | 20 (49%) |
I/I | Not applicable | 1 (2%) |
I/II | Not applicable | 13 (32%) |
IV/otherd or V/otherd | Not applicable | 7 (17%) |
aAge (in months) was different between CF and healthy (Wilcoxon p value = 0.003, r = 0.31, CI = 0.12–0.48).
bGender distribution of healthy and CF was not different (Fisher’s exact test for count data, p value > 0.05).
cEleven of the 15 newborns were recruited for the longitudinal study. Only one sample is currently available from the other four infants.
dKnown class I, II or III PI mutation