Table 1 Baseline characteristics.

From: Body composition changes and clinical outcomes in pediatric cystic fibrosis during 24 months of lumacaftor ivacaftor therapy based on real-world data

 

All patients

2–5 years old

6–11 years old

 ≥ 12 years old

p-value

n

49

15

14

20

 

Number of boys (%)

57

47

50

70

0.315

Age at diagnosis (months)*

8.9 (3.4, 24.2)

11.5 (3.6, 22.0)

6.4 (1.8, 20.1)

10.0 (4.7, 24.9)

0.367

Age at the start of LUM/IVA (years)*

9.3 (5.5, 14.2)

4.6 (3.5, 5.2)

8.5 (7.9, 9.5)

16.2 (13.5, 17.2)

 

Sweat chloride (mmol/L)*

74 (67, 86)

70 (66, 78)

73 (62, 77)

83 (69, 92)

0.154

ppFEV1 (%)*

94 (79, 112)

109 (103, 122)

82 (70, 96)

0.001

ppFVC (%)*

103 (87, 118)

112 (98, 122)

93 (78, 110)

0.037

Fecal elastase (µg/g)

    

1.000

 < 100

39 (98%)

13 (100%)

12 (100%)

14 (93%)

 

 100–200

0 (0%)

0 (0%)

0 (0%)

0 (0%)

 

 > 200

1 (2%)

0 (0%)

0 (0%)

1 (7%)

 

CFRD

4/49 (8%)

0/15 (0%)

0/14 (0%)

4/20 (20%)

0.034

P. aeruginosa colonization

    

0.276

 Never

10 (21%)

3 (20%)

3 (21%)

4 (21%)

 

 Free

19 (40%)

4 (27%)

6 (43%)

9 (47%)

 

 Intermittent

14 (29%)

8 (53%)

3 (21%)

3 (16%)

 

 Chronic

5 (10%)

0 (0%)

2 (14%)

3 (16%)

 
  1. LUM/IVA lumacaftor/ivacaftor, ppFEV1 percentile predicted forced expiratory volume in the first second, ppFVC percentile predicted forced vital capacity, CFRD cystic fibrosis-related diabetes.
  2. *median, interquartile range.
  3. Significant values are in bold.