This is consistent with the observation that CFTR dysfunction primarily affects cells of the immune response including platelets, leukocytes and vascular endothelial cells (Painter et al., 2006; Mattoscio et al., 2010; Del Porto et al., 2011; Sorio et al., 2011; Plebani et al., 2017; Totani et al., 2017), making the pathogenesis of inflammation in CF quite complex. This evidence concerns the gene CFTR and cystic fibrosis.