Servicio de Alergia, Hospital La Paz Health Research Institute (IdiPaz), Biomedical Research Network on Rare Diseases-U754 (CIBERER), Madrid, Spain.
J Allergy Clin Immunol. 2012 Feb;129(2):308-20. doi: 10.1016/j.jaci.2011.11.025. Epub 2011 Dec 24.
There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH).
We sought to elaborate guidelines for optimizing the management of gynecologic/obstetric events in female patients with HAE-C1-INH.
A roundtable discussion took place at the 6th C1 Inhibitor Deficiency Workshop (May 2009, Budapest, Hungary). A review of related literature in English was performed.
Contraception: Estrogens should be avoided. Barrier methods, intrauterine devices, and progestins can be used. Pregnancy: Attenuated androgens are contraindicated and should be discontinued before attempting conception. Plasma-derived human C1 inhibitor concentrate (pdhC1INH) is preferred for acute treatment, short-term prophylaxis, or long-term prophylaxis. Tranexamic acid or virally inactivated fresh frozen plasma can be used for long-term prophylaxis if human plasma-derived C1-INH is not available. No safety data are available on icatibant, ecallantide, or recombinant human C1-INH (rhC1INH). Parturition: Complications during vaginal delivery are rare. Prophylaxis before labor and delivery might not be clinically indicated, but pdhC1INH therapeutic doses (20 U/kg) should be available. Nevertheless, each case should be treated based on HAE-C1-INH symptoms during pregnancy and previous labors. pdhC1INH prophylaxis is advised before forceps or vacuum extraction or cesarean section. Regional anesthesia is preferred to endotracheal intubation. Breast cancer: Attenuated androgens should be avoided. Antiestrogens can worsen angioedema symptoms. In these cases anastrozole might be an alternative. Other issues addressed include special features of HAE-C1-INH treatment in female patients, genetic counseling, infertility, abortion, lactation, menopause treatment, and endometrial cancer.
A consensus for the management of female patients with HAE-C1-INH is presented.
目前针对 C1 抑制剂缺陷（HAE-C1-INH）所致遗传性血管性水肿女性患者的妇科/产科事件管理，仅有数量有限的出版物。
我们旨在详细阐述 HAE-C1-INH 女性患者的妇科/产科事件管理优化指南。
在第六届 C1 抑制剂缺陷研讨会（2009 年 5 月，匈牙利布达佩斯）上进行了圆桌讨论。以英语对相关文献进行了复习。
避孕：应避免使用雌激素。可使用屏障方法、宫内节育器和孕激素。妊娠：减弱雄激素禁忌，在尝试受孕前应停药。人血浆衍生 C1 抑制剂浓缩物（pdhC1INH）是急性治疗、短期预防或长期预防的首选。如果无法获得人血浆衍生 C1-INH，则可使用氨甲环酸或病毒灭活的新鲜冷冻血浆进行长期预防。尚无关于艾替班特、拉那芦肽或重组人 C1-INH（rhC1INH）的安全性数据。分娩：阴道分娩时并发症罕见。分娩前可能无需进行预防，但应备有 pdhC1INH 治疗剂量（20 U/kg）。然而，应根据妊娠期间和先前分娩时的 HAE-C1-INH 症状来处理每例患者。建议在使用产钳或真空抽吸或剖宫产前进行 pdhC1INH 预防。应优选区域麻醉而不是气管内插管。乳腺癌：应避免使用减弱雄激素。抗雌激素可能会加重血管性水肿症状。在这些情况下，阿那曲唑可能是一种替代选择。还讨论了其他问题，包括 HAE-C1-INH 治疗女性患者的特殊特征、遗传咨询、不孕、流产、哺乳、绝经治疗和子宫内膜癌。
提出了 HAE-C1-INH 女性患者管理的共识。
