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<article article-type="research-article" dtd-version="1.3" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">akusherstvo</journal-id><journal-title-group><journal-title xml:lang="en">Obstetrics, Gynecology and Reproduction</journal-title><trans-title-group xml:lang="ru"><trans-title>Акушерство, Гинекология и Репродукция</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2313-7347</issn><issn pub-type="epub">2500-3194</issn><publisher><publisher-name>IRBIS LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.17749/2313-7347.2015.10.1.100-110</article-id><article-id custom-type="elpub" pub-id-type="custom">akusherstvo-39</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ОRIGINAL ARTICLES</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ СТАТЬИ</subject></subj-group></article-categories><title-group><article-title>PREGNANCY AND DELIVERY IN PATIENTS WITH MESENHYMAL DYSPLASIAS (MARFAN SYNDROME, EHLERS-DANLOS SYNDROME, HEREDITARY HEMORRHAGIC TELANGIECTASIA)</article-title><trans-title-group xml:lang="ru"><trans-title>ВЕДЕНИЕ БЕРЕМЕННОСТИ И РОДОВ  У ПАЦИЕНТОК С МЕЗЕНХИМАЛЬНЫМИ  ДИСПЛАЗИЯМИ (СИНДРОМАМИ  МАРФАНА, ЭЛЕРСА-ДАНЛО,  ВРОЖДЕННОЙ ГЕМОРРАГИЧЕСКОЙ  ТЕЛЕАНГИОЭКТАЗИЕЙ)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Радецкая</surname><given-names>Л. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Radetskaya</surname><given-names>L. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., доцент кафедры акушерства и гинекологии медико-профилактического факультета Первого МГМУ им. И.М. Сеченова. Адрес: ул. Трубецкая, 8, стр. 2, Москва, Россия, 119048</p></bio><bio xml:lang="en"><p>MD, docent of the Department of Obstetrics and Gynecology, Faculty of Medical and Preventive Medicine First Moscow Medical Sechenov University. Adress: ul. Trubetskaya, 8, str. 2, Moscow, Russia, 119048</p></bio><email xlink:type="simple">udaeva@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Макацария</surname><given-names>А. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Makatsariya</surname><given-names>A. D.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., член-корреспондент РАН, профессор, заведующий кафедрой акушерства и гинекологии медико-профилактического факультета Первого МГМУ им. И.М. Сеченова. Адрес: ул. Трубецкая, 8, стр. 2, Москва, Россия, 119048. Тел.: +7 (495) 788-58-40.</p></bio><bio xml:lang="en"><p>MD, corresponding member of the Russian Academy of Sciences, Professor, Head of the Department of Obstetrics and Gynecology, Faculty of Medical and Preventive, First Moscow State Medical Sechenov University. Address: ul. Trubetskaya, 8, str. 2, Moskva, Russia, 119048. Tel.: +7 (495) 788-58-40</p></bio><email xlink:type="simple">gemostasis@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ГБОУ ВПО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2016</year></pub-date><pub-date pub-type="epub"><day>05</day><month>06</month><year>2016</year></pub-date><volume>10</volume><issue>1</issue><fpage>100</fpage><lpage>110</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Radetskaya L.S., Makatsariya A.D., 2016</copyright-statement><copyright-year>2016</copyright-year><copyright-holder xml:lang="ru">Радецкая Л.С., Макацария А.Д.</copyright-holder><copyright-holder xml:lang="en">Radetskaya L.S., Makatsariya A.D.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://www.gynecology.su/jour/article/view/39">https://www.gynecology.su/jour/article/view/39</self-uri><abstract><sec><title>Aim</title><p>Aim. To study the pregnancy course and delivery outcome in patients with mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome (EDS), Hereditary hemorrhagic telangiectasia (HHT).</p></sec><sec><title>Material and Methods</title><p>Material and Methods. 56 pregnant women with mesenchymal dysplasias (23 with Marfan syndrome, 22 with Ehlers-Danlos syndrome, 11 with hereditary hemorrhagic telangictasia (HHT) were examined in specialized cardiological maternity house by using physical examination, routine laboratory tests, hemostasiological tests, ECG, echocardiography, MRI, radiology, pulse oximetry.</p></sec><sec><title>Results</title><p>Results. All patients had hemorrhagic complications various localizations; 18 women with Marfan syndrome developed increasing of mitral regurgitation, 10 – aortic insufficiency, 13 – mitral valve insufficiency. 1 patient with Marfan syndrome was died due to aortic dissection in 45 day after delivery. Pulmonary arteriovenous malformations were visualized in 10 patients with HHT; the symptoms of hypoxemia developed in 6 HHT patients and were result of right-to-left. One patient with HHT developed the symptoms of compression of vena cava inferior due to aneurism of truncus brachiocephalicus. 40 patients had abnormal hemostasiological tests (platelet dysfunction – in 38 patients; DIC syndrome – in 31). Cesarean section was performed in 53 patients. Vaginal delivery (3 patients with EDS) was complicated by massive postpartum hemorrhage and deep vaginal tears.</p></sec><sec><title>Conclusions</title><p>Conclusions. Patients with mesenchymal dysplasias have a great risk of different complications, during pregnancy and delivery and require the multidisciplinary care and repeated hemostasiological testing. Cesarean section is the preferred method of delivery in such patients.</p></sec></abstract><trans-abstract xml:lang="ru"><p>Цель – изучение особенностей течения беременности и родов и разработка тактики ведения беременных с мезенхимальными дисплазиями (синдромами Марфана, Элерса-Данло, врожденной геморрагической телеангиоэктазией).</p><sec><title>Материалы и методы</title><p>Материалы и методы. Проведен анализ 56 беременностей у пациенток с мезензимальными дисплазиями, включая «стертые» формы заболеваний: 23 – с синдромом Марфана, 22 – с синдромом Элерса-Данло и 11 – с врожденной геморрагической телеангиоэктазией в возрасте от 18 до 36 лет. Исследование включало ретроспективный анализ и проспективное исследование. Обследование включало в себя сбор анамнеза, клиническое обследование, лабораторные тесты, включая расширенное гемостазиологическое исследование, инструментальные исследования (ЭКГ, ЭхоКГ, рентгенография грудной клетки, КТ, МРТ), молекулярную диагностику синдромов.</p></sec><sec><title>Результаты</title><p>Результаты. Абсолютно у всех пациенток во время беременности отмечалось прогрессирование ранее имевшихся геморрагических проявлений и появление кровотечений новых локализаций. У беременных с синдромом Марфана отмечена высокая частота осложнений со стороны сердечно-сосудистой системы, включавших увеличение степени пролапса митрального клапана (18 беременных), развитие и прогрессирование аортальной недостаточности (10), формирование или прогрессирование недостаточности митрального клапана (13). Одна больная с синдромом Марфана погибла через полтора месяца после родоразрешения от разрыва аорты. Артериовенозные мальформации в легких обнаружены у 10 беременных с синдромом Рендю-Ослера. У шести из них имелись признаки гипоксии вследствие шунтирования крови справа налево. У одной беременной с синдромом Рендю-Ослера во второй половине беременности появились симптомы сдавления верхней полой вены, причиной которого явилось формирование аневризмы плечеголовного ствола. Различные дефекты в системе гемостаза обнаружены у 40 беременных: у 38 нарушение функции тромбоцитор, у 31 – признаки хронического ДВС-синдрома. 53 беременных с мезенхимальными дисплазиями были родоразрешены путем операции кесарево сечение. При родоразрешении через естественные родовые пути (у трех беременных с синдромом Элерса-Данло) отмечались глубокие разрывы мягких тканей промежности и массивные кровотечения.</p></sec><sec><title>Заключение</title><p>Заключение. Беременность и роды у больных с мезенхимальными дисплазиями представляют высокий риск развития осложнений как у матери, так и у плода. Для успешного исхода беременности у таких больных следует рекомендовать мультидисциплинарный подход, а также тщательное динамическое наблюдение с широким спектром лабораторно-инструментальных исследований, включая расширенное гемостазиологическое исследование. Ввиду высокого риска угрожающих жизни осложнений при родоразрешении через естественные родовые пути пациенткам с мезенхимальными дисплазиями показано родоразрешение путем операции кесарево сечение.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>Мезенхимальные дисплазии</kwd><kwd>синдром Марфана</kwd><kwd>синдром Элерса-Данло</kwd><kwd>врожденная геморрагическая телеангиоэктазия (синдром Ослера-Вебера-Рендю)</kwd><kwd>врожденная патология соединительной ткани</kwd><kwd>расслоение аорты у беременных</kwd><kwd>артериовенозные мальформации</kwd></kwd-group><kwd-group xml:lang="en"><kwd>Inherited connective tissue disorders</kwd><kwd>mesenhymal dysplasias</kwd><kwd>Marfan syndrome</kwd><kwd>Ehlers-Danlos syndrome</kwd><kwd>Osler-Weber- Rendu disease</kwd><kwd>Hereditary Hemorrhagic Telangiectasia</kwd><kwd>pregnancy</kwd><kwd>aortic dissection</kwd><kwd>arteriovenous malformations</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Камоева C.В., Савченко Т.Н., Иванова А.В., Абаева Х.А. 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