Clinical characteristics, n
Age, years
Sex, female / male, n
Height, cm
Weight, kg
VOC#, n last year
At least one ACS, n patients
ACS, total number / child
Cerebral vasculopathy*, n
Hydroxyurea / Chronic transfusion, n
|
43
12.6 [10.4; 13.6]
23 / 20
149 [140; 161]
39.0 [33.2; 47.5]
0 [0; 1]
13
0 [0; 1]
17
26 / 12
|
Biological characteristics, n
SS / SB+thalassemia / SC, n
Deficit G6PD, n
Hemoglobin, g/dL
Leucocyte count, x 10+9/L
Reticulocyte count, x 10+9/L
Lactic dehydrogenase, IU/L
|
43
35 / 2 / 6
2
8.5 [8.0; 9.6]
8.14 [7.14; 10.58]
193.2 [148.9; 295.2]
519 [412; 764]
|
PFTs characteristics, n
FEV1, % predicted
FEV1, z-score
FVC, % predicted
FVC, z-score
FEV1/FVC, % predicted
FEV1/FVC, z-score
VA, % predicted $
VA, z-score $
KCO, % predicted
KCO, z-score
DLCO, % predicted $
DLCO, z-score $
KCOcorrected, % predicted
KCOcorrected, z-score
DLCOcorrected, % predicted $
DLCOcorrected, z-score $
DmCO/VA,
mL.min-1.mmHg-1.L-1
Vc/VA, mL/L
|
43
93 [80; 104]
-0.54 [-1.51; 0.34]
98 [85; 104]
-0.13 [-1.22; 0.35]
98 [93; 101]
-0.28 [-1.04; 0.11]
86 [79; 100]
-1.12 [-1.96; 0.03]
110 [99; 118]
0.65 [-0.04; 1.14]
96 [92; 113]
-0.23 [-1.24; 0.76]
158 [152; 173]
3.35 [3.01; 4.70]
138 [120; 167]
2.23 [1.33; 3.34]
17.9 [16.8; 20.0]
25.6 [20.8; 28.1]
|