1-脱氨基-8-D-精氨酸血管加压素治疗血友病A的临床疗效

Clinical effect of desmopressin in treatment of patients with hemophilia A

目的 观察 1 脱氨基 8 D 精氨酸血管加压素(DDAVP)治疗轻中型血友病A患者临床止血效果。方法 血友病A患者 17例 ,重型 3例 ,轻、中型 14例。DDAVP 0 3～ 0 4μg·kg-1,缓慢静脉注射 ,并于注射前及注射后 6 0min、12 0min测定因子Ⅷ∶C活性。结果 ① 3例重型患者无明显疗效 ;14例轻中、型患者使用DDAVP后 ,出血症状均明显减轻 ,关节肌肉血肿处疼痛肿胀减轻、消失。②DDAVP治疗前血浆因子Ⅷ∶C活性平均 5 1%± 4 5 % ,治疗后 6 0、12 0min分别升达 30 8%± 30 2 %、2 5 3 %± 31 5 %。③副作用 :使用DDAVP后 2例出现心慌、恶心 ,1例有面色潮红及球结膜充血。结论 轻、中型血友病A患者使用DDAVP后血浆因子Ⅷ∶C活性均达到有效止血浓度 ,临床出血症状也得到很好控制 ,提示DDAVP在轻、中型血友病A患者有良好的临床疗效 ,建议对轻、中型血友病A患者及早使用DDAVP。

Objective\ To observe the clinical effect of desmopressin(DDAVP) on patients with hemophilia A. Methods\ 17 patients with hemophilia A, comprised of 3 severe cases,14 mild or moderate type cases. DDAVP 03～04 μg·kg -1 body weight was injected intravenously slowly. Coagulative activity of factor Ⅷ in plasma was measured before and after using DDAVP. Results\ Good responses were observed in 14 patients with mild or moderate hemophilia A. The level of FⅧ∶C in plasma was increased significantly after using DDAVP than before (308%±302% vs 51%±45%). 2 patients had nausea and palpitation,1 had conjurictival congestion.Conclusion Markedly clinical effects can be observed in using DDAVP in treatment of patients with mild or moderate severity hemophilia A. Early use of DDAVP in onset of patients with hemophilia A should be recommended.