Semmelweis University, 3rd Department of Internal Medicine, Budapest, Hungary.
Eur J Obstet Gynecol Reprod Biol. 2010 Sep;152(1):44-9. doi: 10.1016/j.ejogrb.2010.05.008. Epub 2010 Jun 11.
The course of hereditary angioedema (HAE) and the efficacy and safety of human C1-INH concentrate were appraised during pregnancy and the postpartum period, in patients with HAE.
Retrospective analysis of clinical data on 118 pregnancies (82 full-term and 36 abortions) in 41 female patients, extracted from the National HAE Registry, medical charts and patient diaries.
HAE attack frequency increases in 48% of pregnancies, whereas 33% of pregnancies were associated with mitigation of clinical signs and 19% of the pregnancies had no influence on the course of HAE, as compared to disease severity seen during the 2-year period preceding the pregnancy. During 46 full-term pregnancies, 26 patients reported attacks; 52% of these occurred in the third trimester. Abdominal attacks are the most common presentation of HAE during pregnancy. Attack number was significantly higher in patients who had sustained their initial attack before 8 years of age. Attack number increased during the third trimester if the fetus was afflicted by HAE. During the postpartum period, attacks occurred in 6/82 pregnancies. Patients received 91 vials of C1-INH concentrate altogether for the relief of acute attacks and for short- or long-term prophylaxis during pregnancy. This therapy was effective in all instances; no adverse effects were observed.
Pregnancy can either aggravate or mitigate edematous attacks, or alternatively, it may have no influence on the severity of the disease. According to our experience, C1-INH concentrate is an effective and safe therapeutic option during pregnancy.
评估遗传性血管性水肿（HAE）患者在妊娠和产后期间的疾病进程以及人 C1-INH 浓缩物的疗效和安全性。
从国家 HAE 登记处、病历和患者日记中提取了 41 名女性患者的 118 例妊娠（82 例足月和 36 例流产）的临床数据进行回顾性分析。
与妊娠前 2 年观察到的疾病严重程度相比，48%的妊娠中 HAE 发作频率增加，33%的妊娠与临床体征减轻相关，19%的妊娠对 HAE 病程无影响。在 46 例足月妊娠中，26 例患者报告发作；其中 52%发生在妊娠晚期。腹部发作是妊娠期间 HAE 最常见的表现。如果患者在 8 岁之前持续发生初始发作，发作次数会显著增加。如果胎儿患有 HAE，则在妊娠晚期发作次数会增加。在 82 例产后妊娠中，有 6 例发生了发作。患者总共接受了 91 瓶 C1-INH 浓缩物用于缓解急性发作以及在妊娠期间进行短期或长期预防。这种治疗在所有情况下均有效，未观察到不良反应。
妊娠可加重或减轻水肿发作，也可能对疾病严重程度无影响。根据我们的经验，C1-INH 浓缩物是妊娠期间有效且安全的治疗选择。
