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Table 5 Comparison of CF+RM with age and gender controlled CFclassic

From: What can the CF registry tell us about rare CFTR-mutations? A Belgian study

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
  1. Data are presented in n (%), Median (IQR) or mean (SD)
  2. p-value: statistical significant if < 0.05 bold
  3. * Two sample t-test
  4. ** Fisher’s exact test
  5. ȸ Fisher’s exact test with continuity correction
  6. ȢBonferroni adjusted threshold P < 0.01
  7. aDays of IV AB (antibiotics): in patients who received IV antibiotics