Triggianese P, Senter R, Petraroli A, Zoli A, Lo Pizzo M, Bignardi D, Di Agosta E, Agolini S, Arcoleo F, Rossi O, Modica S, Greco E, Chimenti M S, Spadaro G, De Carolis C, Cancian M
Rheumatology, Allergology and Clinical Immunology, Department of "Medicina dei Sistemi", University of Rome Tor Vergata, Rome, Italy.
Department of Systems Medicine, University Hospital of Padua, Padua, Italy.
Front Med (Lausanne). 2022 Sep 14;9:930403. doi: 10.3389/fmed.2022.930403. eCollection 2022.
In women with Hereditary Angioedema (HAE) due to C1-inhibitor (C1INH) deficiency (C1INH-HAE), pregnancy counseling and treatment can be challenging. Despite the evidence of the immediate favorable outcome and safety of plasma-derived (pd)C1INH concentrate, there are no data regarding any difference among women who underwent or not pdC1INH during pregnancy or on children with exposure to pdC1INH. The present interview study aimed at analyzing outcome of C1INH-HAE mothers and children according to pdC1INH-exposure during pregnancies.
C1INH-HAE women who experienced at least 1 pregnancy were included from seven centers of the Italian Network for Hereditary and Acquired Angioedema (ITACA). The interview study retrospectively analyzed pregnancies who underwent (group 1) or not (group 2) pdC1INH. The overall goals of the study included immediate and long-term outcomes, in terms of outcomes in the time interval between pregnancy and survey.
A total of 168 pregnancies from 87 included women were analyzed. At term delivery (>37 gestation-week, GW) has been registered in 73.8% of cases, while spontaneous abortion (SA) occurred in 14.2% of cases with a mean GW 7 ± 2. The group 1 including pdC1INH-treated pregnancies comprised a third of the cohort (51/168, time interval 1.5 ± 10.4 yrs), while the group 2 represented 69.6% (117/168, time interval 32.8 ± 14 yrs). The same prevalence of SA occurred when comparing group 1 (11.7%) with group 2 (15.4%) with a similar GW at SA. The group 1 was older at the pregnancy time and younger at the interview than the group 2 ( < 0.01 for both); moreover, the group 1 showed a higher prevalence of cesarean delivery ( < 0.0001). The overall prevalence of obstetrical syndromes was similar between two groups: however, gestational diabetes was described only in pdC1INH-untreated pregnancies. pdC1INH-exposed children ( = 45) did not show differences compared with unexposed ones ( = 99) in neonatal short-term outcomes.
Through appropriate management and counseling, most of C1INH-HAE women undergo successful pregnancy and delivery. For pregnant C1INH-HAE women being treated with pdC1INH, our findings are reassuring and might lead to an improvement of both the knowledge about treatments and the experience of HAE itself.
在因C1抑制物（C1INH）缺乏导致的遗传性血管性水肿（HAE）女性患者中，妊娠咨询和治疗颇具挑战性。尽管有证据表明血浆源性（pd）C1INH浓缩物能带来即时良好疗效且安全，但尚无关于孕期接受或未接受pdC1INH治疗的女性之间，以及暴露于pdC1INH的儿童之间存在差异的数据。本访谈研究旨在根据孕期pdC1INH暴露情况分析C1INH-HAE母亲及其子女的结局。
从意大利遗传性和获得性血管性水肿网络（ITACA）的七个中心纳入至少经历过1次妊娠的C1INH-HAE女性。该访谈研究回顾性分析了接受（第1组）或未接受（第2组）pdC1INH治疗的妊娠情况。研究的总体目标包括妊娠至调查期间的即时和长期结局。
共分析了87名纳入女性的168次妊娠。足月分娩（>37孕周，GW）占73.8%，而自然流产（SA）发生率为14.2%，平均GW为7±2。接受pdC1INH治疗的妊娠组成第1组，占队列的三分之一（51/168，时间间隔1.5±10.4年），第2组占69.6%（117/168，时间间隔32.8±
