THBD and acute respiratory distress syndrome: In addition, plasminogen activator inhibitor-1, soluble thrombomodulin (sTM) and von Willebrand factor-antigen, involved in endothelial injury and dysregulated coagulation, are also implicated in the pathogenesis of adult [17–19] and pediatric [20, 21] ARDS, potentially through microvascular thrombosis contributing to dead space ventilation and organ dysfunction.