Sickle cell disease-related pneumopathy: a bicentric cross-sectional study exploring respiratory function in pediatric drepanocytosis

<bold>Background:</bold> Sickle cell disease (SCD) is an inherited hemolytic anemia in which a mutation in the β-globin gene results in vaso-occlusive crises (VOC) in different organs. The American Thoracic Society recognizes SCD-related pneumopathy as a priority research field. However, its onset and natural history are poorly understood. <bold>Aims and methods:</bold> To cross-sectionally assess the lung function of 17 children with SCD attending two Italian hospitals from May to July 2023. Demographic, clinical and laboratory data were collected, alongside age-specific pulmonary function tests (PFT) and lung ultrasound (LU). Correlations between PFT, LU and parameters of SCD activity were explored, with p<0.05 as threshold. <bold>Results:</bold> Preschooler SCD children (mean age 3.8 years 土 2.13 SD) showed normal lung function, while older children (12.9 years 土 1.66 SD) displayed predominantly restrictive patterns. PFT alterations were associated with age and HbS levels, and also with hospital admission for VOC or acute chest syndrome (p=0.003). 8/17 subjects had pathological LU (b-line score≠0), significantly associated with higher LDH (p=0.01) and life transfusion rate (p=0.004) <bold>Conclusions:</bold> Our findings seem to suggest a gradual lung involvement in SCD, according to disease onset and activity. Upcoming multicenter case-control studies will refine lung function trajectories in SCD, enabling better understanding and earlier management.