Role of the endothelial platelet dysfunction in the genesis of hemorrhages in congenital connective tissue dysplasia

We detected in patients with congenital connective tissue dysplasia (CTD) with hemorrhagic episodes reduced collagen-induced platelet aggregation and von Willebrand factor activity, a trend to a higher thromboxane B(2)/6-keto-prostaglandin F(1 alpha) ratio, and an increase of endothelin-1 level in cases with Marfan's syndrome. These changes indicated on the formation of endothelial platelet dysfunction. More pronounced shifts in the endothelial platelet values in CTD with hemorrhagic episodes were detected in female patients and in patients with multiple localizations of hemorrhages.