deamino-arginine-vasopressin and Behcet-Syndrome

Fibrinolysis dependent on the release of tissue plasminogen activator (t-PA) can only be explored after stimulation eliciting the release of t-PA from endothelial cells. The choice of the stimulus and test assay is of utmost importance in discriminating patients at risk for persistent thrombosis and in identifying acquired or genetic abnormalities of t-PA synthesis or release from endothelial cells in pathologic conditions. The present study was designed to compare the efficacy and reproducibility of the fibrinolytic response to desmopressin acetate (deamino-8-D-argininevasopressin) (DDAVP) by use of various routes of administration with the response to the venous occlusion test. Nine healthy male volunteers were randomly administered intravenous desmopressin acetate, intranasal drops and intranasal spray. The results from tests of euglobulin lysis time, t-PA activity, t-PA antigen levels, and t-PA fast-acting inhibitor (plasminogen activator inhibitor [PAI]) level were compared with those obtained after venous occlusion. The only test that elicited the release of free t-PA activity in the circulation in all volunteers was the intravenous administration of 0.4 microgram/kg desmopressin acetate, and the most reliable test assay was t-PA activity measured in the euglobulin fraction of plasma. Intravenous desmopressin acetate induced the release of large amounts of t-PA in most cases and caused a significant fall in PAI. Other routes of administration of desmopressin acetate, along with venous occlusion, identified many nonresponders who proved to be false negative. The relevance of these data was demonstrated in a study in nine patients with thromboembolic phenomenon or related disorders.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Administration, Intranasal; Adult; Arm; Behcet Syndrome; Deamino Arginine Vasopressin; Female; Hematologic Tests; Humans; Injections, Intravenous; Ligation; Lupus Erythematosus, Systemic; Male; Thrombophlebitis; Tissue Plasminogen Activator; Veins

Vasculitis with central diabetes insipidus is a rare condition which must be recognized for an appropriate management. We report a case of Behçet disease with central diabetes insipidus. A forty seven year old men presented recurring oral and genital ulcers, skin lesions, polyarthralgia. Two years later, he showed right uveitis and central diabetes insipidus without dysfunction of the pituitary gland. Computed tomographic scan showed normal neurohypophysis and pituitary gland. Later on, he presented hemiplegia. The cerebral computed tomographic scan showed hypodense lesions. Central diabetes insipidus should not be systematically searched because it is a rare feature of the Behçet disease.

Topics: Behcet Syndrome; Brain Ischemia; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Hemiplegia; Humans; Magnetic Resonance Imaging; Male; Middle Aged

Recurrent thromboembolic accidents are frequent complications of Behçet's disease. Ten patients were investigated, 5 of them during an acute exacerbation of the disease. Abnormal responses to the venostasis test and to DDAVP (i.v.) were demonstrated in acute cases. Factor VIII activities after DDAVP were increased in all 10 patients, which suggests that the response of the vascular endothelium to the stimulus was normal and that a fibrinolytic system inhibitor may be present in the plasma of some patients.

Topics: Adolescent; Adult; Arginine Vasopressin; Behcet Syndrome; Blood Coagulation Tests; Child; Deamino Arginine Vasopressin; Factor VIII; Female; Fibrinolysis; Humans; Infusions, Parenteral; Male; Middle Aged; Serum Globulins; Veins

Suppression of the fibrinolytic system is a well-known phenomenon in patients with Behcet's disease regardless of whether they present thrombotic complications. This finding has been related to impaired production and/or release of plasminogen activators from the vascular endothelium. In previous studies a diminished release of PF4 upon heparin stimuli was observed in plasma from patients with Behcet's disease and interpreted as an additional indicator for endothelial cell dysfunction. In the present investigations, 12 patients and 10 healthy volunteers received DDAVP infusions and euglobulin clot lysis time, factor VIII activities and 6-keto-PGF1 alpha levels in plasma were repeatedly determined before and after infusions. At different times following DDAVP infusion, euglobulin clot lysis time was significantly longer and levels of F.VIII R:Ag were lower in patients than in normals. F. VIII:C activity increased in both groups, whereas no changes were seen in the plasma levels of 6-Keto-PGF1 alpha either in normals or in patients. It is concluded that the disseminated damage of endothelial tissue associated with Behçet's disease correlates with multiple endothelial cell dysfunctions and subsequent hemostatic abnormalities.

Topics: 6-Ketoprostaglandin F1 alpha; Adult; Arginine Vasopressin; Behcet Syndrome; Deamino Arginine Vasopressin; Endothelium; Factor VIII; Female; Fibrinolysis; Humans; Male; Middle Aged; Plasminogen Activators