akusherstvoObstetrics, Gynecology and ReproductionАкушерство, Гинекология и Репродукция2313-73472500-3194IRBIS LLC10.17749/2070-4968.2015.9.4.074-085akusherstvo-184Research ArticleCLINICAL LECTURESКЛИНИЧЕСКИЕ ЛЕКЦИИPREGNANCY AND HERITABLE CONNECTIVE TISSUE DISORDERS (MARFAN SYNDROME, EHLERS DANLOS SYNDROME, OSLER-WEBER-RENDU DISEASE)БЕРЕМЕННОСТЬ И МЕЗЕНХИМАЛЬНЫЕ ДИСПЛАЗИИ(СИНДРОМЫ МАРФАНА,ЭЛЕРСА-ДАНЛО, РЕНДЮ-ОСЛЕРА)РадецкаяЛ. С.RadetskayaL. S.

к.м.н., доцент кафедры акушерства и гинекологии медико-профилактического факультета

MD, docent of the Department of Obstetrics and Gynecology

udaeva@gmail.comГБОУ ВПО Первый МГМУ им. И.М. Сеченова Минздрава РФ, МоскваРоссияFirst Moscow State Medical Sechenov University of the Ministry of Health Russian FederationRussian Federation201520062016947485Copyright © Radetskaya L.S., 20162016Радецкая Л.С.Radetskaya L.S.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.gynecology.su/jour/article/view/184

The article describes some inherited disorders (Marfan syndrome, Ehlers-Danlos syndrome, Osler-Weber-Rendu disease) that affects the connective tissue with prominent manifestations in the skeletal, ocular, and cardiovascular systems during the pregnancy. The present article aims to provide an overview of these rare but very important hereditary disorders. Diagnosing of these disorders can be difficult because the symptoms can vary significantly from person to person. There are clinical criteria and molecular methods of diagnostics. The pathogenesis of these inherited connective tissue disorders has not been fully elucidated. Pregnancy can be dangerous in such patients due to the high risk of lifetreating complications. Cesarean section is preferred in women with inherited connective tissue disorders.

Цель данной статьи - привлечь внимание клиницистов к такой редко встречающейся, но имеющей чрезвычайно важное клиническое значение патологии, как мезенхимальные дисплазии (синдромы Марфана, Элерса-Данло, Рендю-Ослера), в частности, особенностям течения данных заболеваний во время беременности. Патология соединительной ткани может охватывать различные органы и системы, симптомы одного и того же заболевания могут варьировать у различных пациентов, что вызывает значительные трудности в диагностике. Описаны клинические диагностические критерии и методы молекулярной диагностики заболеваний. Патогенез мезенхимальных дисплазий остается до конца не изученным. Беременность пациенток с мезенхимальными дисплазиями сопряжена с высоким риском угрожающих жизни осложнений. Предпочтительным методом родоразрешения таких пациенток является родоразрешение путем операции кесарево сечение.

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