Sánchez Palacios A, Schamann Medina F, García Marrero J A
Allergy Unity, Hospital Insular, Las Palmas de Gran Canaria, Spain.
Allergol Immunopathol (Madr). 1998 Jul-Aug;26(4):195-8.
Three cases of clinical angioedema are reported in which the etiopathogenetic involvement of qualitative and quantitative complement disorders was demonstrated. The first patient had a functional deficit in C1 inhibitor, the second had a decrease in CH50, and the third, a reduction in the C1q, C3, and C4 fractions. The cases are interesting because of occasional difficulties in the causal diagnosis, the severity of the symptoms, which can include laryngeal edema, and, finally, the favorable outcome achieved with correct medication, which did not include antihistamines or steroids. The clinical picture of hereditary angioedema is characterized by the familial occurrence of the process, although this apparently was absent in these cases. Two patients experienced laryngeal edema and none had abdominal manifestations. The treatment of choice for angioedema of these characteristics is antifibrinolytic agents, which achieve good results in 70% of the patients. Epsilon-aminocaproic acid and tranexamic acid inhibit the formation of plasmin and fragments of the Hageman factor, thus inhibiting kallikrein and bradykinin production. The androgens danazol and stanazolol have been used since the 1970s, and stanazolol proved to be very effective in two of our patients.
报告了3例临床血管性水肿病例，其中证实了定性和定量补体紊乱的病因学参与。第一例患者C1抑制剂存在功能缺陷，第二例患者CH50降低，第三例患者C1q、C3和C4组分减少。这些病例很有趣，原因在于病因诊断偶尔存在困难、症状严重（可包括喉头水肿），以及最终通过正确用药（不包括抗组胺药或类固醇）取得了良好疗效。遗传性血管性水肿的临床表现特点是该病程具有家族性发生情况，尽管这些病例中显然不存在这种情况。两名患者出现了喉头水肿，且均无腹部表现。对于具有这些特征的血管性水肿，首选治疗药物是抗纤维蛋白溶解剂，70%的患者使用该药物可取得良好效果。ε-氨基己酸和氨甲环酸可抑制纤溶酶和Hageman因子片段的形成，从而抑制激肽释放酶和缓激肽的产生。自20世纪70年代以来一直使用雄激素达那唑和司坦唑醇，司坦唑醇在我们的两名患者中被证明非常有效。
