Number, (%)
|
11 (25)
|
11 (25)
|
16 (36.4)
|
6 (13.6)
| | |
Age, yr ± SD
|
12.18 ± 3.09
|
10.55 ± 2.46
|
11.81 ± 3.02
|
13.17 ± 2.79
|
10.89-12.65
|
0.32
|
Shwachman-Kulczycki score ± SD
|
55.0 ± 11.18
|
81.82 ± 3.37
|
82.19 ± 7.52
|
90.83 ± 9.17
|
71.89-81.06
|
0.000
|
Pseudomonas aeruginosa positive, n (%)
|
9 (81.8)
|
4 (36.4)
|
7 (43.8)
|
1 (16.7)
| |
0.04
|
Hospitalised, n (%)
|
9 (81.8)
|
4 (50)
|
2 (14.3)
|
0 (0)
| |
0.001
|
FEV1 ± SD
|
48.45 ± 18.02
|
83.27 ± 16.57
|
89.00 ± 12.91
|
105.33 ± 14.03
|
72.23-87.09
|
0.000
|
Health-related quality of life (CFQ-R) Patients
| | | | | | |
Physical functioning ± SD
|
55.18 ± 29.33
|
81.25 ± 11.13
|
80.12 ± 21.38
|
78.94 ± 18.81
|
66.56-81.12
|
0.02
|
Emotional functioning ± SD
|
70.08 ± 9.20
|
76.42 ± 12.41
|
79.12 ± 12.41
|
79.17 ± 7.28
|
72.69-79.67
|
0.19
|
Social limitations ± SD
|
68.11 ± 12.88
|
73.54 ± 14.85
|
72.47 ± 16.61
|
63.23 ± 13.59
|
65.75-74.88
|
0.50
|
Eating disturbances ± SD
|
68.69 ± 20.98
|
70.00 ± 32.31
|
90.97 ± 16.84
|
90.74 ± 10.92
|
73.06-87.67
|
0.02
|
Treatment burden ± SD
|
65.66 ± 22.47
|
70.00 ± 26.22
|
84.72 ± 12.75
|
81.48 ± 16.72
|
69.59-82.34
|
0.07
|
Body image ± SD
|
47.47 ± 28.15
|
73.33 ± 27.32
|
80.56 ± 22.77
|
87.04 ± 14.77
|
62.74-79.889
|
0.005
|
Respiratory symptoms ± SD
|
53.57 ± 21.27
|
74.52 ± 16.53
|
67.83 ± 14.50
|
79.76 ± 12.39
|
62.02-73.45
|
0.01
|
Digestive symptoms ± SD
|
90.00 ± 14.29
|
83.33 ± 22.38
|
81.95 ± 23.96
|
83.34 ± 18.26
|
78.04-90.75
|
0.80
|
Health-related quality of life (CFQ-R) Caregivers
| | | | | | |
Physical functioning ± SD
|
46.76 ± 33.12
|
91.20 ± 10.60
|
87.26 ± 14.07
|
67.90 ± 2.13
|
66.96-85.90
|
0.000
|
Emotional functioning ± SD
|
76.67 ± 16.33
|
84.07 ± 5.72
|
76.95 ± 18.17
|
71.11 ± 13.88
|
73.06-83.61
|
0.53
|
Vitality ± SD
|
66.67 ± 16.33
|
76.67 ± 14.25
|
69.99 ± 11.55
|
60.00 ± 6.67
|
64.89-74.90
|
0.27
|
Eating disturbances ± SD
|
64.58 ± 31.42
|
74.07 ± 39.18
|
79.17 ± 21.47
|
66.67 ± 0.00
|
62.59-83.24
|
0.02
|
Treatment burden ± SD
|
44.45 ± 19.70
|
64.19 ± 14.46
|
65.74 ± 18.02
|
70.37 ± 42.07
|
52.67-68.16
|
0.10
|
Body image ± SD
|
61.11 ± 26.56
|
76.54 ± 23.20
|
85.19 ± 19.15
|
68.52 ± 27.40
|
66.56-83.79
|
0.16
|
Weight ± SD
|
29.17 ± 37.53
|
40.74 ± 32.39
|
86.11 ± 17.16
|
100.0 ± 0.00
|
46.62-74.22
|
0.000
|
School performance ± SD
|
66.67 ± 30.86
|
86.42 ± 17.37
|
77.77 ± 23.69
|
85.19 ± 25.66
|
69.34-86.91
|
0.39
|
Health perception ± SD
|
50.00 ± 31.43
|
69.14 ± 22.07
|
70.37 ± 19.73
|
70.37 ± 16.97
|
56.23-73.63
|
0.26
|
Respiratory symptoms ± SD
|
58.13 ± 21.81
|
79.80 ± 18.01
|
80.89 ± 10.31
|
79.889 ± 19.14
|
68.01-81.45
|
0.03
|
Digestive symptoms ± SD
|
75.00 ± 19.47
|
74.69 ± 13.92
|
63.89 ± 15.80
|
70.37 ± 23.13
|
64.22-76.41
|
0.42
|