USE OF DDAVP TO IMPROVE HEMOSTASIS IN GAUCHER'S DISEASE

这项研究是为了确定高雪氏症患者是否 疾病和血小板减少症可以安全地接受侵入性手术 (即肝脏活检)在药物De氨基D-Arg的覆盖下- 血管加压素(DDAVP)，从而避免接触血液产品。这个 所有患者均在术前接受CBC、凝血筛查 (PT、PTT、TT、纤维蛋白原)、LFT‘s、BUN和血清肌酐，并评估 单纯出血时间法一次止血。所有见过的病人 研究参数(血小板计数50,000但小于100,000；出血 10分钟。)然后接受标准的DDAVP(0.3ug/kg静脉输注) 超过30分钟)。L在完成手术一小时后重复出血时间 DDAVP输液。所有表现出短缩的患者 出血时间少于或等于10分钟。然后接受了肝脏移植 活组织检查。血管性血友病因子与血小板的随访评价 所有接受DDAVP治疗的患者随后均获得功能 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.