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Figure 1

Changes in forced expiratory volume in 1 second (FEV1) with treatment in the CP (A) and CP + device (B) groups (individual patient data). Changes in forced vital capacity (FVC) with treatment in the CP (C) and CP + device (D) groups (individual patient data).
Changes in forced expiratory volume in 1 second (FEV1) with treatment in the CP (A) and CP + device (B) groups (individual patient data). Changes in forced vital capacity (FVC) with treatment in the CP (C) and CP + device (D) groups (individual patient data).

Figure 2

Change in maximal expiratory flow at 25% of forced vital capacity (MEF25) with treatment in the CP (A) and CP + device (B) groups (individual patient data).
Change in maximal expiratory flow at 25% of forced vital capacity (MEF25) with treatment in the CP (A) and CP + device (B) groups (individual patient data).

Pulmonary function tests.

CP CP + device
Baseline Before discharge Mean change from baseline to discharge p-value Baseline/ Before discharge Mean change from baseline to discharge p-value
LCI:
Mean ± SD 12.3±4.0 11.5±3.8 –0.8±2.0 0.030 14.4±3.8

n=18 in the CP + device group.

13.7±4.1

n=18 in the CP + device group.

–0.7±2.4

n=18 in the CP + device group.

NS
% change –5.0±16.3 -4.4±13.5

n=18 in the CP + device group.

LCI z-score
Mean ± SD 19.2±22.1 18.1±24.6 -1.1±7.9 NS 29.0±27.5

n=18 in the CP + device group.

23.9±27.5

n=18 in the CP + device group.

–5.1±14.8

n=18 in the CP + device group.

NS
% change –5.3±49.5 –10.1±21.8

n=18 in the CP + device group.

FEV1, % predicted:
Mean ± SD 76.0±20.6 83.3±19.9 7.3±12.0 0.007 66.7±19.2 69.6±23.5 9.0±9.1 <0.001
% change 11.5±17.5 14.3±14.2
FVC, % predicted
Mean ± SD 87.8±18.8 91.1±16.9 3.3±6.9 0.030 77.7±18.6 83.2±20.4 5.5±8.1 0.003
% change 4.4±7.7 7.1±11.2
FEV1/FVC, %
Mean ± SD 86.8±10.2 91.6±8.2 4.8±9.0 0.003 77.0±14.1 82.2±16.5 5.0±6.0 <0.001
% change 6.6±14.1 7.7±7.8
FEV1/FVC z-score
Mean ± SD -1.58±1.07 -1.06±0.97 0.52±0.95 0.007 -2.42±1.142 -1.93±1.28 0.49±0.64 0.001
% change -18.0±68.3 - 47.4±120.4
MEF25, % 52.0±32.9 65.9±34.7 13.9±29.6 NS 29.4±20.7 41.8± 35.5 12.4±18.8 0.006
MEF50, % 73.5±34.9 87.6±35.5 14.1±29.5 0.022 48.3±32.2 59.0±33.1 10.7±18.0 0.008
MEF75, % 75.7±25.1 90.7±29.1 15.0±23.1 0.004 66.0±27.9 78.3±31.2 12.3±18.2 0.003
MEF25-75, % 58.7±28.2 73.0±28.8 14.3±27.1 0.006 38.0±24.9 48.0±31.2 10.0±14.1 <0.002

Characteristics of the study population.

Characteristic CP (n=24) CP + device (n=24) p-value
Age, years 13 (12–16) 14 (13–17) NS
Male, n (%) 16 (66.7) 7 (29.2) <0.01
Height, cm 161.0±12.3 159.3±14.7 NS
BMI, kg/m2 18.9±2.5 18.2±5.9 NS
BMI z-score –0.2±0.8 –0.9±0.9 <0.05
LCI 12.3±4.0 14.4±3.8

n=18 in the CP + device group.

<0.05
LCI z-score (7.312.9 –19.5) (10.915.0 –50.0)

n=18 in the CP + device group.

NS
FEV1, % predicted 76.0±20.6 66.7±19.2 <0.05
FEV1, L 2.4±0.9 1.9±0.8 <0.05
FVC, % predicted 87.8±18.8 77.7±18.6 <0.05
FVC, L 3.2±1.1 3.0±1.2 <0.05
FEV1/FVC, % 86.8±10.2 77.0±14.1 <0.01
FEV1/FVC z-score -1.58±1.07 -2.42±1.14 <0.02
MEF25-75, % predicted 58.7±28.2 38.0±24.9 <0.01 NS
Pseudomonas aeruginosa, n (%) 8 (33) 13 (54)
F508del homozygous, n (%) 11 (46) 14 (58) NS
Pancreatic insufficiency, n (%) 23 (96) 23 (96) NS
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