Opferkuch W, Echternacht K, Gronemeyer W, Hammar C, Jaeger U, Niemczyk H, Rieger C
Immun Infekt. 1980;8(2):56-60.
Sera of 333 patients showing clinical symptoms of "Quincke edema" have been tested for their deficiency of C1 esterase inhibitor. The diagnosis "Hereditary Angio Neurotic Edema" (HANE) could be stated in 39 cases, i.e. 11.9%. Clinical manifestations consisted of peripheral edema (80%), abdominal colics (77%), and facial edema (72%). Edema of the larynx have been observed in 51% of the cases. 10 patients have been treated with purified C1 esterase inhibitor during the attack as a substitutional therapy, and 10 patients received danazol during their attackfree intervals. Both of these treatments were therapeutically successful.
对333例有“昆克水肿”临床症状患者的血清进行了C1酯酶抑制剂缺乏检测。39例（即11.9%）可诊断为“遗传性血管性水肿”（HANE）。临床表现包括外周水肿（80%）、腹部绞痛（77%）和面部水肿（72%）。51%的病例观察到喉部水肿。10例患者在发作期间接受了纯化的C1酯酶抑制剂替代治疗，10例患者在无发作间期接受了达那唑治疗。这两种治疗在治疗上均取得成功。
