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.,肝活组织检查） 脱氨基-D-精氨酸-加压素（DDAVP），从而避免暴露于血液 产品.所有患者均接受了CBC的术前筛查， 凝血筛查（PT、PTT、TT、纤维蛋白原）、LFT'S、BUN和血清 肌酐，并通过Simplate出血时间评估主要止血。 所有符合研究参数（血小板计数50，000，但 小于100，000;出血时间为10分钟），然后接受标准 DDAVP（0.3ug/kg，静脉滴注30 min）。一个流血的时刻， DDAVP输注完成后1小时重复。的所有患者 显示出血时间缩短至小于或等于10分钟 然后接受了肝脏活检。von Willebrand的随访评价 随后获得所有患者的凝血因子和血小板功能， 接收DDAVP以与对DDAVP的响应相关联。

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 min) then received a standard infusion of DDAVP (0.3ug/kg IV over 30 min). A bleeding time was then repeated 1 hour after completion of the DDAVP infusion. All patients who demonstrated a shortening of bleeding time to less than or equal to 10 min 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.