Both agents can be administered intravenously or orally [1,2]. For oral use of epsilon aminocaproic acid, a dose of 60–80 mg Kg−1 3–4 times a day is given, and for tranexamic Doxorubicin acid, a dose of 15–25 mg Kg−1 3–4 times a day is recommended. A mouth wash with tranexamic acid (10 mL of a 5% solution) 4–6 times a day can also be beneficial for controlling gingival bleeding. Desmopressin is a synthetic analogue of the antidiuretic hormone – vasopressin that increases the plasma concentrations of von Willebrand factor (VWF) and factor VIII and has been used successfully in patients with mild von Willebrand disease and mild haemophilia A [3]. The mechanism of desmopressin action

has not been elucidated. In 1984, Kobrinsky et al. [4] showed that desmopressin is also effective in patients with inherited platelet dysfunctions. In that study, the bleeding time was shortened in all patients examined and haemostasis was secured in 8 patients undergoing surgery albeit with the aid of epsilon aminocaproic acid administration. Since then, several small series of desmopressin-treated patients with variable inherited platelet dysfunctions have been

reported [5–10] and comprehensively reviewed [11,12]. Although it is questionable whether shortening of the bleeding time means adequate haemostasis during surgery, it is notable that in a subset of patients with inherited platelet dysfunctions and shortening of bleeding time following desmopressin administration, no excessive bleeding occurred during surgery when Dabrafenib it was preceded by desmopressin infusion [5,6,9,10]. Entities for which unequivocal evidence indicates that bleeding time shortens after desmopressin include delta-storage pool disease, disorders of granule secretion, unexplained prolonged bleeding time, May-Hegglin anomaly, signal transduction disorders and thromboxane receptor anomaly [12,13]. Cediranib (AZD2171) Equivocal evidence was provided for BSS, Hermansky-Pudlak syndrome and arachidonate metabolism defects [11,12]. Of nine patients with GT, only one exhibited shortening of the

bleeding time after desmopressin infusion [12]. Desmopressin at a dose of 0.3 μG Kg−1 (but not exceeding a total dose of 20 μG) is usually administered intravenously in 50 mL saline over 30 min. Peak levels of VWF are usually obtained 30 min after infusion. When desmopressin is used for surgery, strict timing should be coordinated with the surgeon. Side effects can occur sometimes and include tachycardia, hypotension, facial flushing and headache. Fluid retention and severe hyponatremia with seizures can occur and hence fluid intake should be restricted for 24 h after desmopressin infusion. Several studies, but not all, have indicated that desmopressin infusion confers a risk of arterial thrombosis [11]. Consequently, treatment by esmopressin should be cautiously considered in elderly patients and in patients with cardiovascular disease.