VWF and cystic fibrosis: Increased circulating levels of von Willebrand factor (vWF) and tissue plasminogen activator (tPA), indicative of endothelial damage and altered hemostasis (Romano et al., 2001), and reduced flow-mediated dilation of the brachial artery, which was associated with more severe airway disease and symptomatic of reduced NO availability (Poore et al., 2013), were reported in CF.