Table 1: Patients clinical characteristic.

VariableMedian (range) or number (%) value
StableExacerbation

Number of subjects1315
Female6 (46%)7 (46%)1.000
Age (mean)10.4 (7-15)13.1 (9-18)0.018
F508 del homozygous7 (54%)6 (40%)0.458
F508del heterozygous4 (30%)5 (33%)0.864
3 (13%)0.177
BMI (median)16.6 (14.4-21.0)17.3 (14.03-19.5)0.890
Pancreatic insufficiency10 (77%)13 (87%)0.488
Diabetes3 (0.2%)0.087
Chronic P. aeruginosa infection4 (30%)8 (53%)0.219
Chronic S. aureus infection10 (76%)13 (87%)0.450
FEV1 at admission (% pred.)100.0 (92.0-126.0)69.5 (56.5-87.0)<0.0001
FVC at admission (% pred.)98.0 (89.0-111.0)75.5 (69.5-88.0)<0.0001
TLC (% pred.)118.5 (112.0-124.0)120.0 (108.5-128.5)0.278
RV (% pred.)187.5 (168.0-205.0)237.5 (201.0-296.0)<0.0001
RV/TLC (% pred.)161.5 (136.0-176.0)194.5 (181.5-220.0)<0.0001
Shwachman-Kulczycki scale85.0 (85.0-90.0)67.5 (60.0-77.5)<0.0001
Brasfield score21.0 (18.0-23.0)15.0 (13.0-20.0)<0.001