Abstract
Background: Chronic loss of lung function was described in SCD patients (pts), even if not fully characterized in children. Aim: to explore lung function in children with SCD, through the assessment of resistance measurements and ventilation patterns. Methods: spirometry, FOT, IOS and N2MBW were performed in 15 stable pediatric pts with SCD, F 46.67%, Caucasian 13.33%, aged 12 ±3.8 years, mean HbS 66.36 ±17.44, ICS/LABA therapy in 20%, ICS therapy in 13.33%. Results: A mild restrictive pattern was found (FEV1 86.96 ±15.91 %pred, FVC 75.47 ±26.62%pred, MMEF75-25 86.76 ±21%pred). Lung Clearance Index (LCI) 2.5% was elevated (7.91 ±2.26, 120%pred), as well as normalized acinar slope index Sacin (0.12 ±0.14, 220%pred). IOS measured an increase in resistance (R) at 5 Hz (119.37 [101.68-137.79] %pred) while FOT showed an increase in R at 8 Hz (118.05 [101.87-140.29] %pred) and decrease in reactance(X) at 8 Hz. Expiratory R inversely correlated with MMEF75-25 (r -0.6, p 0.03). Pts Conclusions: IOS and FOT may help to depict and monitor early lung function changes in children with SCD. N2MBW did not correlate here with spirometry, FOT or IOS, thus it may be considered to add a novel and complementary insight in functional status of children with SCD, through the representation of ventilation inhomogeneity.
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