Characteristics First evaluation
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]