medbioМедико-биологические проблемы жизнедеятельностиMedical and Biological Problems of Life Activity2074-2088Республиканский научно-практический центр радиационной медицины и экологии человека10.58708/2074-2088.2026-3(39)-64-74medbio-551Research ArticleКЛИНИЧЕСКАЯ МЕДИЦИНАCLINICAL MEDICINEБеременность, роды и послеродовый период у женщин с наследственным ангиоотеком с дефицитом C1-ингибитора: обзор современных подходов к ведению и анализ данных опроса белорусских пациентовPregnancy, delivery, and the postpartum period in women with hereditary angioedema with C1 inhibitor deficiency: a review of current management approaches and analysis of survey data from Belarusian patienhttps://orcid.org/0000-0002-9696-3949ГурьяноваИ. Е.GuryanovaI. E.

г. Минск

guryanovairina85@gmail.comЖаранковаЮ. С.ZharankovaYu. S.

г. Минск

marukovich85@mail.ruОвсяникИ. В.AusianikI. V.

г. Гродно

ausianik.iryna@gmail.comЛебедьВ. И.LebedzV. I.

г. Гродно

vikintosh@gmail.comhttps://orcid.org/0009-0002-4093-1267СолнцеваА. В.SolntsavaA. V.

г. Минск

ang_solntseva@mail.ruГУ «РНПЦ детской онкологии, гематологии и иммунологии»BelarusОО «Помощь пациентам с наследственным ангиоотеком»Belarus202601112026036474Copyright © Гурьянова И.Е., Жаранкова Ю.С., Овсяник И.В., Лебедь В.И., Солнцева А.В., 20262026Гурьянова И.Е., Жаранкова Ю.С., Овсяник И.В., Лебедь В.И., Солнцева А.В.Guryanova I.E., Zharankova Y.S., Ausianik I.V., Lebedz V.I., Solntsava A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://medbio.ejournal.by/jour/article/view/551

Наследственный ангиоотек, обусловленный дефицитом или функциональной недостаточностью C1-ингибитора (НАО-C1-INH), требует особого внимания при ведении беременности в связи с непредсказуемым влиянием гормональных изменений на активность заболевания, риском развития потенциально тяжёлых приступов и необходимостью выбора безопасной терапии для матери и плода. В статье систематизированы современные данные литературы и актуальные рекомендации по ведению женщин с НАО-C1-INH во время беременности, родов и грудного вскармливания. Обобщение опубликованных данных демонстрирует, что НАО не является противопоказанием к реализации репродуктивных планов. Дополнительно представлены результаты анкетирования 8 белорусских женщин с НАО-C1-INH, имевших в общей сложности 19 беременностей, 14 из которых завершились родами. В данной когорте течение заболевания характеризовалось выраженной вариабельностью: увеличение частоты приступов отмечалось в 6 из 14 (42,9%) беременностей, практически полное прекращение приступов — в 5 из 14 (35,7%), отсутствие существенных изменений — в 3 из 14 (21,4%), при этом характер течения мог различаться при последовательных беременностях. В одном случае абдоминальный приступ НАО был расценён как острая хирургическая патология, что привело к выполнению аппендэктомии на 26-й неделе беременности и последующему прекращению её развития. Большинство родов (12 из 14; 85,7%) произошли через естественные родовые пути, а приступы НАО в первые 72 часа после родоразрешения отмечались в 3-х из 14 случаев (21,4%). Представленные в статье актуальные подходы к терапии и профилактике НАО-C1-INH могут служить практическим руководством для специалистов, участвующих в ведении пациентов с НАО, способствуя своевременной диагностике и лечению приступов, рациональному выбору терапии и улучшению качества жизни пациентов

Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) requires particular attention during pregnancy because of the unpredictable effects of hormonal changes on disease activity, the risk of potentially severe attacks, and the need to select treatment that is safe for both the mother and the fetus. This article summarizes current evidence from the literature and up-to-date recommendations for the management of women with C1-INH-HAE during pregnancy, delivery, and breastfeeding. The available evidence demonstrates that HAE is not a contraindication to pursuing reproductive plans. In addition, we present the results of a survey of 8 Belarusian women with C1-INH-HAE who had a total of 19 pregnancies, 14 of which resulted in delivery. Disease course in this cohort was highly variable: an increase in attack frequency was reported in 6/14 (42,9%) of pregnancies, near-complete cessation of attacks in 5/14 (35.7%), and no substantial change in 3/14 (21,4%); moreover, the course of the disease could differ between successive pregnancies. In one case, an abdominal HAE attack was misdiagnosed as an acute surgical condition, resulting in appendectomy at 26 weeks of gestation, followed by pregnancy loss. Most deliveries occurred vaginally (12/14, 85,7%), while HAE attacks within the first 72 hours after delivery occurred in 3/14 cases (21,4%). The current approaches to the treatment and prophylaxis of C1-INH-HAE presented in this article may serve as a practical guide for healthcare professionals involved in the management of patients with HAE, facilitating timely diagnosis and treatment of attacks, rational selection of therapy, and improvement of patients’ quality of life.

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The authors declare that there are no conflicts of interest present.