P183 Effect of targeted therapy on structural and functional lung changes in cystic fibrosis (CF) in children

We assessed changes in parameters (CT scan and PFTs) before targeted therapy children with CF during treatment to determine their dynamics. examined 12 (4 boys, 8 girls) treated elexacaftor+/tezacaftor+/ivacaftor (TRICAFTA®), age at the start of therapy: Me = 15.7 ± 1.5 (min 12.3 - max 17.3); mutations (in 5 patients delF508/delF508, rest: delF508/W1282x, F508del/1259insA, delF508/ del2 3-21 kb,c, F508del/N1303 K, 2184 insA, R334W/W1282R). The results PFTs CT lungs were 5–7 months after therapy. was performed on a MasterScreen Jaeger (Germany). General Electric Discovery CT750 HD (USA). Retrospectively quantitative assessment structural carried out scores according Brody Scale. Statistica 6.0 software package (StatSoft®) using Wilcoxon criteria revealed statistically significant differences (p < 0.05) between dynamics pairs). Significant found for following treatment: (FEV1) %D, 46.6 [30.6;66.4] vs 67.5 [40.5;85] 0.01); (MOS75) %D 29 [10.5;36.4] 38.6 [13.0;54.0] 0.002); Mucous plugging (Mp) score, 25 [19.5;30] 13 [11.0;20.5] 0.004); Peribronchial thickening (Pth) score 38.9 [22.6; 43.4] 17.6 [15.5;24.4] 0.006); (total score) 142.5 [104.1;165.4] 106.4 [97.1;138.8] 0.005). There also trend towards less areas increased airiness, but not (Me 15 [10.5;20.5] versus 14.5 [7.5;20.5] 0.06)). use resulted improved lung function, particular increase FEV1 as well improvements CT, namely lower total due reduction Mp Pth, bronchiectasis.