Table 5 Comparison of CF_+RM with age and gender controlled CF_classic

Number (n)

70

140

Male

37 (52.9%)

74 (52.9%)

1.0000

Adults

46 (65.7%)

88 (62.9%)

0.6846

 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 chloride^Ȣ

(mmol/L)

64 (91,4%)

117 (83.6%)

96.3 (63.7–107.0)

104.0 (97.1–115.0)

< 0.0001

 Missing

6 (8,6%)

23 (16.4%)

0.1198

 0- ≤ 30 mmol/L

3 (4.3%)

0 (0%)

0.0360^ȸ

 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 AB^a

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