血液疾患における口腔出血管理に関する研究 : 第20報DDAVPによる女性血友病A患者の止血管理について

概要

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Haemophilia A is a bleeding disorder that usually seen in males and is inherited as a sex-linked recessive trait. The disorder has been reported in females in several families with male haemophiliacs. However about 20% of female haemophiliacs are described as sporadic type.<BR>On the other hand, resently a synthetic vasopressin DDAVP (1-deamino-8-D-arginine vasopressin) was used for haemostatic control of haemophiliacs. The haemostatic mechanism of DDAVP in haemophilia A and von Willebrands disease is understandable because of the increment of factor VII activity.<BR>Scaling was performed in a female haemophilia A (VII: C 7%) and then DDAVP (0.4 μg/kg) was administered for haemostatic treatment. DDAVP infusion (iv) induced the increment of factor VII activity. This procedure resulted in effective haemostasis without factor VII replacement therapy.