This includes vaccination against hepatitis B, avoidance of non-steroidal anti-inflammatory drugs, preservation of dental hygiene, correction of iron deficiency anemia and prenatal diagnosis when the mutation is known. (ii) Topical hemostatic measures. These can involve compression with gauze MK-8669 cell line soaked with tranexamic acid, fibrin sealants containing tranexamic acid, acrylic splints for dental extraction, and packing for nose bleeds. (iii) Antifibrinolytic agents (epsilon aminocaproic acid or tranexamic acid). Such agents are useful for prevention of bleeding

following minor surgery, and can be employed as adjuncts of other treatment modalities such as recombinant factor VIIa (rFVIIa), 1-desamino-8D-arginine vasopressin (DDAVP), and platelet transfusion. Patients should be guided to start oral treatment with one of the antifibrinolytic agents whenever troublesome bleeding occurs and thereafter seek medical attention if necessary. Antifibrinolytic agents are essential for Quebec platelet syndrome. (iv) Management by DDAVP. This agent increases

the plasma concentrations of von Willebrand factor (VWF) and factor VIII. The mechanism of DDAVP action has been attributed to increased adhesiveness of platelets to the subendothelial matrix, and to augmented platelet aggregation at high shear rate resulting in shortening of bleeding time www.selleckchem.com/products/Roscovitine.html [40]. Several small series of DDAVP-treated patients with variable inherited platelet dysfunctions have been reported. Entities for which unequivocal evidence indicates that bleeding time shortens after DDAVP include delta-storage pool diseases, disorders of granule secretion, signal transduction disorders, thromboxane MCE公司 receptor deficiency, May-Hegglin anomaly and patients with unexplained prolonged bleeding time. Equivocal evidence was provided for BSS, HPS and arachidonate metabolism

defects. Patients with GT do not respond to DDAVP [41]. Side effects of DDAVP administration include tachycardia, hypotension, facial flushing, headache, severe hyponatremia with seizures and arterial thrombosis. (v) rFVIIa. GT patients have been treated for bleeding episodes by rFVIIa with partial success [42,43]. The mechanism by which rFVIIa arrests bleeding is probably related to increased thrombin generation by a tissue factor-independent process, enhanced adhesion of platelets to extracellular matrix and restoration of platelet aggregation [44–46]. The use of rFVIIa in patients with inherited platelet dysfunction has not been examined by randomized controlled studies. Among 59 GT patients in an international survey, 75% of 108 spontaneous bleeding episodes and 94% of 34 surgical procedures were manageable by rFVIIa. However, two patients who received a high dose of rFVIIa developed pulmonary embolism and a ureteric clot, respectively [42]. (vi) Female hormones.