USE OF DDAVP TO IMPROVE HEMOSTASIS IN GAUCHER'S DISEASE

本研究旨在确定戈谢病患者是否 疾病和血小板减少症可以安全地进行侵入性手术 (i.e.,肝活检）在药物脱氨基-D-精氨酸的覆盖下， 血管加压素（DDAVP），从而避免暴露于血液制品。 的 所有患者均接受了CBC、凝血筛查的术前筛查 (PT、PTT、TT、纤维蛋白原）、LFT、BUN和血清肌酐，并评估 通过简化出血时间进行初步止血。 所有符合 研究参数（血小板计数50，000但小于100，000;出血 时间10分钟）。DDAVP（0.3ug/kg）静脉滴注 超过30分钟）。 然后在完成后1小时重复出血时间 DDAVP注射液。 所有显示短缩的患者 出血时间小于或等于10分钟。然后接受了肝脏移植 活检 血管性假血友病因子和血小板的随访评价 随后在所有接受DDAVP的患者中获得功能， 与对DDAVP的反应相关。

This study was undertaken to determine whether patients with Gaucher's disease and thrombocytopenia could safely undergo invasive procedures (i.e., liver biopsy) under the coverage of the drug Deamino-D-Arginine- Vasopressin (DDAVP) and thereby avoid exposure to blood products. The patients all received pre-procedure screening with CBC, Coagulation screen (PT, PTT, TT, Fibrinogen), LFT's, BUN & serum creatinine, and assessment of primary hemostasis by Simplate bleeding time. All patients who met the study parameters (platelet count of 50,000 but less than 100,000; bleeding time of 10 mins.) then received a standard infusion of DDAVP (0.3ug/kg IV over 30 mins.). A bleeding time was then repeated l hour after completion of the DDAVP infusion. All patients who demonstrated a shortening of bleeding time to less than or equal to 10 mins. then underwent a liver biopsy. Follow-up evaluation of von Willebrand factor and platelet function were subsequently obtained on all patients who received DDAVP to correlate with the response to DDAVP.