达那唑对遗传性血管水肿的长期预防性治疗作用  

Long-term Prophylactic Effect of Danazoi for Hereditary Angioedema Patients

作　　者：支玉香[1] 张宏誉[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院变态反应科,北京100730

出　　处：《中华临床免疫和变态反应杂志》2007年第2期175-179,共5页Chinese Journal of Allergy & Clinical Immunology

摘　　要：目的观察达那唑对遗传性血管水肿(HAE)长期预防性治疗的疗效及副作用.同时探讨达那唑的作用机制。方法回顾性分析1987～2006年于北京协和医院诊断并接受达那唑治疗的45例 HAE 患者的疗效、副作用及治疗前后生化指标的改变。结果 80.0%的患者能以200mg/d 或200mg/d 以下的剂量维持以达到良好控制。在治疗开始阶段,达那唑剂量较大时,C1抑制物(C1 INH)浓度和 C4水平较治疗前明显升高(P<0.01),C4达到了正常范围,但 C1 INH 未能达到正常值。当达那唑减至维持量后,二者水平均下降,C1 INH与治疗前无差异(P>0.05),而 C4仍高于治疗前(P<0.05)。达那唑副作用主要表现为肝功能的异常和雄性激素的作用表现,但耐受良好,且减至维持量后副作用轻微。结论达那唑可有效用于 HAE 的长期预防性治疗,副作用轻微,耐受性好。其作用机制可能与 C1 INH 抑制作用的再分配有关,确切的机制仍需进一步研究。Objective To evaluate the efficacy and side effect of danazol in the long-term prophylaxis of hereditary angioedema （HAE） and investigate the mechanism of danazol in it. Methods By reviewing the HAE patients who were diagnosed in Peking Union Medical College Hospital during 1987-2006, the efficacy and side effects of danazol were observed and the levels of C1 inhibitor （C1 INH） and C4 antigen before and after treatment were analyzed in 45 patients who received danazol for long-term prophylaxis. Results Danazol was effective in most of the patients （80.0%） with the maintenance dosage of 200 mg/d or less than 200 mg/d. At the beginning when large dosage of danazol was used, the level of C1 INH antigen increased slightly （P〈 0.01）, but it did not reach the normal level; while the level of C4 increased significantly （P〈 0.01） and reached the normal level. But when the dosages were reduced to the maintenance dosage, both of them decreased. The level of C1 INH decreased to the level before treatment, while the level of C4 was still higher than that of beginning （P 〈 0.05）. The main side effects included liver function damage and the androgen effect. Conclusions Danazol is effective and well tolerated in HAE long-term prophylaxis. The role of danazol may be associated with the redistribution of the C1 INH effect.

关 键 词：遗传性血管水肿 达那唑 C1抑制物 

分 类 号：R596[医药卫生—内科学]