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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">10.1234/XXXX-XXXX-2015-1-25-31</article-id><article-id custom-type="elpub" pub-id-type="custom">medgen-4</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 АТРЕЗИИ ПИЩЕВОДА (ПО ДАННЫМ МОНИТОРИНГА ВПР)</article-title><trans-title-group xml:lang="en"><trans-title>DESCRIPTIVE EPIDEMIOLOGY OF ESOPHAGEAL ATRESIA (DATA OF BIRTH DEFECTS MONITORING)</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, Москва, ул. Талдомская, д. 2; e-mail: </p><p> </p></bio><email xlink:type="simple">ndemikova@pedklin.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>Выдрыч</surname><given-names>Ю. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Vydrych</surname><given-names>Ju. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>119991, Москва, ул. Трубецкая, д. 8, стр. 2</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Подольная</surname><given-names>М. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Podolnaya</surname><given-names>V. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Минздрава России, 125412, Москва, ул. Талдомская, д. 2</p></bio><email xlink:type="simple">ndemikova@pedklin.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Лапина</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Lapina</surname><given-names>A. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>125412, Москва, ул. Талдомская, д. 2</p></bio><email xlink:type="simple">ndemikova@pedklin.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>Асанов</surname><given-names>А. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Asanov</surname><given-names>A. Ju.</given-names></name></name-alternatives><bio xml:lang="ru"><p>119991, Москва, ул. Трубецкая, д. 8, стр. 2</p></bio><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Обособленное структурное подразделение «Научно-исследовательский клинический институт педиатрии» ГБОУ ВПО РНИМУ им. Н.И. Пирогова Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Clinical Institute of Pediatrics of Pirogov Russian National Research Medical University, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ГБОУ ВПО Первый МГМУ им. И.М. Сеченова Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.M. Sechenov First Moscow State Medical University, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Обособленное структурное подразделение «Научно-исследовательский клинический институт педиатрии» ГБОУ ВПО РНИМУ им. Н.И. Пирогова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Clinical Institute of Pediatrics of Pirogov Russian National Research Medical University, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2015</year></pub-date><pub-date pub-type="epub"><day>20</day><month>12</month><year>2015</year></pub-date><volume>14</volume><issue>1</issue><fpage>25</fpage><lpage>31</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Демикова Н.С., Выдрыч Ю.В., Подольная М.А., Лапина А.С., Асанов А.Ю., 2015</copyright-statement><copyright-year>2015</copyright-year><copyright-holder xml:lang="ru">Демикова Н.С., Выдрыч Ю.В., Подольная М.А., Лапина А.С., Асанов А.Ю.</copyright-holder><copyright-holder xml:lang="en">Demikova N.S., Vydrych J.V., Podolnaya V.A., Lapina A.S., Asanov A.J.</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/4">https://www.medgen-journal.ru/jour/article/view/4</self-uri><abstract><p>Представлены результаты эпидемиологического исследования редкого врождённого порока развития — атрезии пищевода (АП). Данные получены на основе мониторинга ВПР в течение 13-летнего периода (с 2000 по 2012 гг.) в 30 регионах РФ. Обобщённая оценка частоты АП за весь период наблюдения составила 1,96 (95% CI 1,87—2,06) на 10 000 рождений, или</p><p>1 случай на 5099 рождений. Частота порока в течение всего периода наблюдений оставалась стабильной. Среди всех случаев порока 57,3% составляли АП со свищом и 42,7% АП без свища. В 68,2% случаев АП встречалась изолированно, а в 31,8% случаев в сочетании с пороками развития других органов и систем. Определены относительные риски АП. Показано, что риск АП выше для детей женщин старшей возрастной группы: RR 1,51 (95%С11,25—1,84); для новорождённых мужского пола (RR 1,22 (95% CI 1,08—1,38) и для новорождённых с массой тела ниже 3000 г (RR 4,82 (95% CI 4,24—5,48).</p></abstract><trans-abstract xml:lang="en"><p>The paper presents the results of epidemiological studies of rare congenital malformation — esophageal atresia (EA). Data derived from birth defects monitoring during 2000—2012 in 30 regions of the Russian Federation. The total prevalence of AP for the entire observation period was 1.96 (95% C11.87—2.06) per 10 000 births or 1 case per 5099 births. The EA prevalence during the entire observation period remained stable. EA with tracheoesophageal fistula (TEF) represents 57.3% of the cases. In 68.2% of cases EA was isolated anomaly and associated malformations were present in 31.8%. Relative risk for EA was higher for the older maternal age group (RR is 1.51, 95% CI 1.25—1.84); for male (RR 1.22, 95% CI 1.08-to 1.38) and for infants with a birth weight less than 3,000 g (RR 4.82, 95% CI 4.24-5.48).</p><p> </p></trans-abstract><kwd-group xml:lang="ru"><kwd>врождённые пороки развития</kwd><kwd>атрезия пищевода</kwd><kwd>эпидемиология</kwd><kwd>частота</kwd></kwd-group><kwd-group xml:lang="en"><kwd>congenital malformation</kwd><kwd>esophageal atresia</kwd><kwd>epidemiology</kwd><kwd>prevalence</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">Казанцева Л.З., Клембовский А.И., Демикова Н.С. и др. Инструкция по описанию фенотипа детей с врожденными пороками развития. — МЗ РФ, 2001. — 22 с.</mixed-citation><mixed-citation xml:lang="en">Казанцева Л.З., Клембовский А.И., Демикова Н.С. и др. 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