Girl with mild vwF. Previous history of heavy bleeding during tooth extraction. Prophylaxis before tooth extraction:
Ans: A (Desmopressin) Ref: Harrison's Principles of Internal Medicine, 18th edn Explanation:Treatment of von Willebrand DiseaseThe mainstay of treatment for type 1 VWD is 1-deamino-8-d- arginine vasopressin (DDAVP, or desmopressin), which results in release of VWF and FVIII from endothelial stores.DDAVP can be given intravenously or by a high concentration intranasal spray (1.5 mg/mL).The peak activity when given intravenously is approximately 30 minutes, while it is 2 hours when given intranasally.The usual dose is 0.3 g/kg intravenously or 2 squirts (1 in each nostril) for patients >50 kg (1 squirt for those <50 kg).It is recommended that patients with VWD be tested with DDAVP to assess their response before using it.It can be used for procedures with minor to moderate risk of bleeding. It is usually given every 12-24 hours.Less frequent dosing may result in less tachyphylaxis, which occurs when synthesis cannot compensate for the released stores.The major side effect of DDAVP is hyponatremia due to decreased free water clearance.Some patients with types 2A and 2M VWD respond to DDAVP such that it can be used for minor procedures,For the other subtypes, for type 3 disease, and for major procedures requiring longer periods of normal hemostasis, VWF replacement can be given.Virally inactivated VWF-containing factor concentrates are thought to be safer than cryoprecipitate as the replacement product.Antifibrinolytic therapyo Aminocaproic acid or Tranexamic acid.o It is an important therapy, either alone or in an adjunctive capacity, particularly for the prevention or treatment of mucosal bleeding.o These agents are particularly useful in prophylaxis for dental procedures, with DDAVP for dental extractions and tonsillectomy, menorrhagia and prostate procedures,o It is contraindicated in the setting of upper urinary tract bleeding, due to the risk of ureteral obstruction.
Desmopressin
Blood transfusion
Fresh frozen plasma
Cryoprecipitate
Bleeding and Thrombotic Disorders