Table 5.
Comparison of CF+RM with age and gender controlled CFclassic
| CCharacteristics | CF+RM | CFclassic | p-value |
|---|---|---|---|
| Number (n) | 70 | 140 | |
| Male | 37 (52.9%) | 74 (52.9%) | 1.0000 |
| Adults | 46 (65.7%) | 88 (62.9%) | 0.6846 |
| Mutation-distribution | |||
| F508del homozygous | 0 (0.0%) | 129 (92.1%) | - |
| F508del heterozygous | 42 (60.0%) | 0 (0.0%) | - |
| other | 28 (40.0%) | 11 (7.9%) | < 0.0001 |
| Age (y) | 27.6 (11.9–37.3) | 26.1 (13.2–33.7) | |
| 25.9 (15.0) | 24.3 (13.3) | 0.5406 | |
| Age at diagnosis (y) | 70 (100%) | 140 (100%) | |
| 3.7 (0.3–18.3) | 0.3 (0.1–2.0) | < 0.0001 | |
| Sweat chlorideȢ
(mmol/L) |
64 (91,4%) | 117 (83.6%) | |
| 96.3 (63.7–107.0) | 104.0 (97.1–115.0) | < 0.0001 | |
| Sweat chlorideȢ | |||
| Missing | 6 (8,6%) | 23 (16.4%) | 0.1198 |
| 0- ≤ 30 mmol/L | 3 (4.3%) | 0 (0%) | 0.0360ȸ |
| 31- ≤ 60 mmol/L | 11 (15,7%) | 0 (0%) | < 0.0001 ȸ |
| > 60 mmol/L | 50 (71,4%) | 117 (83.6%) | 0.0398 |
| FEV1%pred | 59 (84.3%) | 123 (87.9%) | |
| 76.7 (57.8–95.9) | 68.4 (48.1–85.6) | 0.0166* | |
| 76.8 (25.4) | 67.4 (24.1) | ||
| BMI-Z score (Cachera) | 65 (92.9%) | 145 (97.9%) | |
| − 0.3 (− 1.0–0.4) | − 0.5 (− 1.1–0.1) | 0.2059 | |
| Patients with IV AB | 25 (35.7%) | 71 (50.7%) | 0.0414 |
| Days of IV ABa | 21.0 (11.0–48.0) | 18.0 (14.0–42.0) | 0.5690 |
| Pancreatic insufficiency | 39 (55.7%) | 137 (97.9%) | < 0.0001 |
| Chronic PA | 17 (24.3%) | 61 (43.6%) | 0.0093 |
| CF liver disease | 3 (4.3%) | 8 (5.7%) | 0.7551** |
| CFRD | 14 (20.0%) | 32 (22.9%) | 0.8432 |
Data are presented in n (%), Median (IQR) or mean (SD)
p-value: statistical significant if < 0.05 bold
* Two sample t-test
** Fisher’s exact test
ȸ Fisher’s exact test with continuity correction
ȢBonferroni adjusted threshold P < 0.01
aDays of IV AB (antibiotics): in patients who received IV antibiotics