HAEMATE P(R) IN CHILDREN WITH VON WILLEBRANDS DISEASE

In our center, 289 children with von Willebrand's disease (vWD) have b een diagnosed since 1982. The majority of cases (n = 198) were congeni tal vWD whereas 91 patients suffered from vWD induced by valproate (VP A). We overview bleeding episodes in 45 children and 64 operative proc edures Bleedings requiring therapeutic intervention. The aim of therap eutic Operations and prophylactic procedures in vWD is correcting the hemostatic disorder and normalization of bleeding time. This can be ac hieved by application of Haemate P leading to an elevation of plasma l evels of von Willebrand parameters together with normalization of blee ding time. In patients with vWD type I, DDAVP will be preferred if con traindications can be excluded and efficacy has been shown. Severe ble eding complications could be prevented in a total of 50 surgical proce dures in children with vWD type I by prophylactic treatment with DDAVP or Haemate P. Two children initially treated with DDAVP had to be sub stituted with Haemate P in the follow-up because of continuous bleedin g. In type IIa and type III vWD as well as in VPA-induced vWD, the use of Haemate P was essential for sufficient hemostasis in all bleeding and operations. We conclude that Haemate P provides effective bleeding prophylaxis and treatment in all types of vWD except platelet-type.