title: Secretory response of the vessel wall to DDAVP and venous occlusion in von Willebrand's disease.
abstract: The aim of this study was to compare the secretory response of the vascular wall in vivo to DDAVP (i.v. 0.3 microgram/kg, 30 min) and to venous occlusion (VO, 20 min) in control healthy subjects, patients with von Willebrand's disease type I (vWd I) and patients with von Willebrand's disease type III (vWd III). In controls (n = 10) and vWd I (n = 12), DDAVP induced a 2 to 3-fold rise in plasma von Willebrand factor antigen (vWf: Ag), factor VIII coagulant activity (VIII: C) and tissue--type plasminogen activator antigen (t-PA:Ag). VO was less effective in increasing vWf: Ag and VIII:C but produced a greater rise in t-PA:Ag. Large increments (over 10-fold) were observed in plasmin-alpha 2-antiplasmin complexes following both stimuli. In vWd III (n = 10), DDAVP and VO failed to increase vWf:Ag, VIII:C and t-PA:Ag. No significant changes in plasmin-alpha 2-antiplasmin complexes were observed in this group. Moreover, the baseline t-PA:Ag values were significantly lower in vWd III (2.17 +/- 1.13 ng/ml) than in controls (4.84 +/- 1.97 ng/ml, p < 0.001). A significant increase in urokinase--type plasminogen activator antigen (u-PA:Ag) was found only in controls after VO. Neither controls nor patients with vWd showed any changes in plasma fibronectin levels following DDAVP. The low t-PA:Ag results and the abnormal fibrinolytic response to DDAVP and VO in patients with severe (type III) vWd indicate that their endothelial cell abnormality is more extensive than the defect in the synthesis or release of vWf.
authors.affiliation: Laboratory of Blood Coagulation and Hemostasis, Institute of Hematology and Blood Transfusion, Warsaw.
authors.forename: K
authors.initials: K
authors.lastname: Bykowska
authors.forename: M
authors.initials: M
authors.lastname: Letowska
authors.forename: J
authors.initials: J
authors.lastname: Sabliński
authors.forename: B R
authors.initials: BR
authors.lastname: Binder
authors.forename: M
authors.initials: M
authors.lastname: Kopeć
authors.forename: S
authors.initials: S
authors.lastname: Lopaciuk
mesh_terms: D000328:Adult; D003250:Constriction; D003894:Deamino Arginine Vasopressin / Q000494:pharmacology* / Q000627:therapeutic use; D004730:Endothelium, Vascular / Q000187:drug effects* / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010960:Plasminogen Activators / Q000378:metabolism; D011956:Receptors, Cell Surface / Q000378:metabolism; D055293:Receptors, Urokinase Plasminogen Activator; D014680:Veins / Q000503:physiopathology; D014842:von Willebrand Diseases / Q000188:drug therapy / Q000503:physiopathology*
chemical_list: C524898:PLAUR protein, human; D011956:Receptors, Cell Surface; D055293:Receptors, Urokinase Plasminogen Activator; D010960:Plasminogen Activators; D003894:Deamino Arginine Vasopressin