Khalife H, Riethmuller D, Roth P, Koeberle P, Schaal J P, Maillet R
Clinique Universitaire de Gynécologie, d'Obstétrique et de la Reproduction, CHRU, Besançon.
J Gynecol Obstet Biol Reprod (Paris). 1996;25(6):559-67.
Hereditary angioneurotic edema is a rare disease (250 cases currently identified in France). It is the most frequent among hereditary deficiencies of the complement system. This severe disease is fatal in 15-20% of the patients before 40 years of age. Death can be prevented if correct steps are taken in case of even minor trauma, which often trigger acute, potentially fatal, episodes. Danazol is used as preventive therapy before surgery and C1-esterase inhibitor or fresh frozen plasma is given for acute episodes. In case of obstetrical trauma, C1-esterase inhibitors should be used either as prophylaxy or as treatment. Fresh frozen plasma is used if C1-esterase inhibitors are not available. We report here our experience with three patients with known hereditary angioneurotic edema. Gynecology surgery was required in two.
遗传性血管性水肿是一种罕见疾病（目前在法国已确诊250例）。它是补体系统遗传性缺陷中最常见的一种。这种严重疾病在40岁之前的患者中有15%至20%会致命。如果在即使是轻微创伤的情况下采取正确措施，往往可以预防死亡，因为轻微创伤常常引发急性、可能致命的发作。达那唑在手术前用作预防性治疗，急性发作时给予C1酯酶抑制剂或新鲜冰冻血浆。对于产科创伤，C1酯酶抑制剂应用作预防或治疗。如果没有C1酯酶抑制剂，则使用新鲜冰冻血浆。我们在此报告我们对三名已知患有遗传性血管性水肿患者的治疗经验。其中两名患者需要进行妇科手术。
