Desmopressin in inherited disorders of platelet function.

摘要: Summary.  Following the first clinical use in haemophilia and von Willebrand disease 1977, synthetic analogue of vasopressin 1-deamino-8-d-arginine (DDAVP, desmopressin) was successfully employed for management a series bleeding disorders, including congenital acquired defects platelet function. In this setting, few haemostatic approaches are available and, particular severe major invasive procedures, transfusion concentrates is first-choice treatment. Therefore, DDAVP (and remains) an attractive therapeutic alternative, being well tolerated, cost-saving, administrable at home (by intranasal or subcutaneous concentrated formulations) particular, enabling avoidance blood product exposition related risks (allergic reactions, transmitted infections). Despite three decades use, cellular mechanisms effects remain poorly known excellent results reported some case have not been strengthened by rigorous trials, hampered rarity heterogeneity these disorders. However, experience more than evidence-based medicine reserved established place to inherited This review will focus data open issues setting.