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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="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">medgen</journal-id><journal-title-group><journal-title xml:lang="ru">Медицинская генетика</journal-title><trans-title-group xml:lang="en"><trans-title>Medical Genetics</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2073-7998</issn><publisher><publisher-name>Publishing House «Genius Media» LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.25557/2073-7998.2023.05.32-39</article-id><article-id custom-type="elpub" pub-id-type="custom">medgen-2289</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="ru"><subject>ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL RESEARCH</subject></subj-group></article-categories><title-group><article-title>Описательная эпидемиология множественных врожденных пороков развития по данным мониторинга ВПР за 2011-2020 годы</article-title><trans-title-group xml:lang="en"><trans-title>Descriptive epidemiology of multiple congenital malformations according to monitoring data for 2011-2020</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>Демикова</surname><given-names>Н. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Demikova</surname><given-names>N. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>125412, г. Москва, ул. Талдомская, д. 2</p><p>125993, г. Москва, ул. Баррикадная, д.2/1, стр.1</p></bio><bio xml:lang="en"><p>2 Taldomskaya st., Moscow, 125412</p><p>2/1 Barrikadnaya st., Moscow, 125993</p></bio><email xlink:type="simple">ns_d@rambler.ru</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>Путинцев</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Putintsev</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>125412, г. Москва, ул. Талдомская, д. 2</p></bio><bio xml:lang="en"><p>2 Taldomskaya st., Moscow, 125412</p></bio><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Научно-исследовательский клинический институт педиатрии и детской хирургии имени академика Ю.Е. Вельтищева ФГАОУ ВО РНИМУ им. Н. И. Пирогова Минздрава России; ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Министерства здравоохранения РФ</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University; Russian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Научно-исследовательский клинический институт педиатрии и детской хирургии имени академика Ю.Е. Вельтищева ФГАОУ ВО РНИМУ им. Н. И. Пирогова Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2023</year></pub-date><pub-date pub-type="epub"><day>27</day><month>07</month><year>2023</year></pub-date><volume>22</volume><issue>5</issue><fpage>32</fpage><lpage>39</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Демикова Н.С., Путинцев А.Н., 2023</copyright-statement><copyright-year>2023</copyright-year><copyright-holder xml:lang="ru">Демикова Н.С., Путинцев А.Н.</copyright-holder><copyright-holder xml:lang="en">Demikova N.S., Putintsev A.N.</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.medgen-journal.ru/jour/article/view/2289">https://www.medgen-journal.ru/jour/article/view/2289</self-uri><abstract><sec><title>Введение</title><p>Введение. В системе эпидемиологического мониторинга врожденных пороков развития (ВПР) множественные ВПР (МВПР) считаются более чувствительными индикаторами негативного влияния окружающей среды и выявления тератогенов по сравнению с изолированными пороками развития. Это обуславливает необходимость изучения эпидемиологии этой группы пороков. Цель исследования – оценка частоты и динамики МВПР в регионах РФ за период с 2011 по 2020 гг., а также анализ структуры МВПР и оценка факторов риска.</p></sec><sec><title>Методы</title><p>Методы. Представлены данные эпидемиологического мониторинга ВПР 23 региональных регистров РФ за период с 2011 по 2020 гг. Общее число зарегистрированных случаев ВПР составило 116705, число МВПР – 8637. Общее число рождений составило 4379889. Частоты рассчитывались на 10000 рождений.</p></sec><sec><title>Результаты</title><p>Результаты. Обшая частота МВПР составила 19,72 на 10000 рождений. Отмечаются межрегиональные колебания частоты группы МВПР. За 10 лет наблюдений отмечается увеличение частоты МВПР с 17,67 до 22,77 на 10000. Доля индуцированных прерываний беремености значимо выше в группе МВПР по сравнению с изолированными ВПР (39,2% и 18% соответственно). Новорожденные с МВПР чаще рождаются с низкой массой тела, с небольшим преобладанием мужского пола (1,5М:1Ж). В структуре пороков развития чаще всего встречаются ВПР сердечно-сосудистой, костно-мышечной и нервной систем.</p></sec><sec><title>Заключение</title><p>Заключение. МВПР представляют собой важную подгруппу редких заболеваний, включенных в системы мониторинга пороков развития. Создание стандартизированной базы данных по МВПР позволит осуществить анализ динамики частот этой группы пороков развития в разных регионах РФ и эффективно решать задачи мониторинга ВПР по выявлению тератогенных факторов.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. In the congenital malformations epidemiological monitoring system, multiple congenital malformations (MCM) are considered to be more sensitive indicators of the negative impact of the environment and the detection of teratogens compared to isolated malformations. So the study of the epidemiology of this group of defects is necessary. The aim of the research is to assess the prevalence and dynamics of MCM in the regions of the Russian Federation for the period from 2011 to 2020, as well as to analyze the structure of MCM and assess risk factors.</p></sec><sec><title>Materials</title><p>Materials. Data from epidemiological monitoring of CM from 23 regional registers of the Russian Federation for the period from 2011 to 2020. The total number of registered cases of congenital malformations was 116,705, the number of MCM cases was 8,637. The total number of births was 4,379,889. The frequencies were calculated per 10,000 births.</p></sec><sec><title>Results</title><p>Results. The overall prevalence of MCM was 19.72 per 10,000 births. There are interregional fluctuations in the prevalence of the MCM group. Over 10 years of observation, there has been an increase in the prevalence of MCM from 17.67 to 22.77 per 10,000. The proportion of induced abortions is significantly higher in the MCM group compared to isolated CMs (39.2% and 18%, respectively). Newborns with MCM are more often born with low body weight, with a slight male predominance (1.5M:1F). In the structure of malformations, congenital malformations of the cardiovascular, musculoskeletal and nervous systems are most common.</p></sec><sec><title>Conclusion</title><p>Conclusion. MCM are an important subgroup of rare diseases included in malformation monitoring systems. The creation of astandardized high-quality database on MCM will make it possible to analyze the dynamics of the prevalence of multiple malformations in different regions of the Russian Federation and effectively solve the tasks of monitoring CMs to identify teratogenic factors.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>множественные врожденные пороки развития</kwd><kwd>эпидемиология</kwd><kwd>мониторинг ВПР</kwd></kwd-group><kwd-group xml:lang="en"><kwd>multiple congenital malformations</kwd><kwd>epidemiology</kwd><kwd>monitoring</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">Martínez-Frías M.L. Approaches to the analysis of infants with multiple congenital anomalies. Am J of Medical Genetics. 2001; 101: 33-35.</mixed-citation><mixed-citation xml:lang="en">Martínez-Frías M.L. Approaches to the analysis of infants with multiple congenital anomalies. 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