Universidade Federal do Paraná, Curitiba, PR, Brazil.
Centro Universitário Autônomo do Brasil, Curitiba, PR, Brazil.
Rev Bras Ginecol Obstet. 2020 Dec;42(12):845-848. doi: 10.1055/s-0040-1718955. Epub 2020 Dec 21.
To verify the efficacy of short-term prophylaxis for vaginal or cesarean section childbirth with plasma-derived C1-inhibitor concentrate in pregnant women. They should have hereditary angioedema (HAE) and normal plasma C1-inhibitor.
Case report of pregnant women diagnosed with HAE with normal C1-inhibitor who had been treated with intravenous C1-inhibitor concentrate for prophylaxis of angioedema attacks when hospitalized for delivery. The exon 9 of the Factor 12 () genotyping gene was performed by automatic sequencing in all patients.
Three cases of pregnant women with HAE with normal serum level of C1-inhibitor are reported. The genetic test detected the presence of a pathogenic mutation in the gene. Deliveries occurred uneventfully and patients had no HAE symptoms in the following 72 hours.
C1-inhibitor concentrate could be useful to prevent angioedema attacks during and after delivery.
验证在患有遗传性血管性水肿（HAE）且血浆 C1 抑制剂正常的孕妇中，使用血浆衍生的 C1 抑制剂浓缩物进行阴道或剖宫产分娩短期预防的疗效。
对因分娩住院时接受静脉注射 C1 抑制剂浓缩物预防血管性水肿发作而被诊断为 HAE 且 C1 抑制剂正常的孕妇进行病例报告。对所有患者进行 12 号因子（）基因外显子 9 的基因分型，采用自动测序。
报告了 3 例血清 C1 抑制剂正常的 HAE 孕妇。基因检测发现 基因存在致病性突变。分娩过程顺利，患者在接下来的 72 小时内没有出现 HAE 症状。
C1 抑制剂浓缩物可用于预防分娩期间和分娩后发生血管性水肿发作。
