perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2022-67-3-39-46perinatology-1650Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESЭпидемиологическая характеристика врожденных пороков развития: данные регистра Московской областиEpidemiological characteristics of congenital malformations: data from the Moscow Region registerhttps://orcid.org/0000-0001-8117-3816ЗаяеваЕ. Е.ZayaevaE. E.

 Заяева Елизавета Евгеньевна — асп. кафедры медицинской генетики; врач-генетик медико-генетического отделения Московского областного научно-исследовательского института акушерства и гинекологии

Москва

 Moscow 

zayaevae@gmail.comhttps://orcid.org/0000-0002-5649-0534АндрееваЕ. Н.AndreevaE. N.

 Андреева Елена Николаевна — к.м.н., доц. курса пренатальной диагностики; зав. медико-генетическим отделением 

101000 Москва, ул. Покровка, д. 22а 

 Moscow 

https://orcid.org/0000-0003-0623-0301ДемиковаН. С.DemikovaN. S.

 Демикова Наталия Сергеевна — д.м.н., доц., зав. кафедрой медицинской генетики; гл. науч. сотр. Центра информационных технологий и мониторинга

125993 Москва, ул. Баррикадная, д. 2/1, стр. 1;

125412 Москва, ул. Талдомская, д. 2 

 Moscow 

ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России; ГБУЗ МО «Московский областной научно-исследовательский институт акушерства и гинекологии»РоссияRussian Medical Academy of Continuous Professional Education; Moscow Regional Research Institute of Obstetrics and GynecologyRussian FederationФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России; ОСП «Научно-исследовательский клинический институт педиатрии им. академика Ю.Е. Вельтищева» ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава РоссииРоссияRussian Medical Academy of Continuous Professional Education; Veltischev Research and Clinical Institute for Pediatrics at the Pirogov Russian National Research Medical UniversityRussian Federation2022090820226733946Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20222022Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/1650

Цель настоящей работы — анализ эпидемиологической картины врожденных пороков развития в разных популяциях в сравнении с данными регионального регистра врожденных пороков развития Московской области. Даны краткая характеристика и результаты работы международных организаций, осуществляющих сбор и анализ данных о частоте и структуре врожденных пороков развития — EUROCAT (European network of population-based registries for the epidemiological surveillance of congenital anomalies) и ICBDSR (The International Clearinghouse for Birth Defects Surveillance and Research). Для стран, в которых отсутствуют мониторинговые системы учета врожденных пороков развития, разработана Всемирная база данных врожденных заболеваний Modell (MGDb), которая дает возможность расчета предполагаемых популяционных частот врожденных пороков развития в этих регионах. По данным международных регистров, наиболее часто встречаются врожденные пороки развития сердечно-сосудистой, мочеполовой, центральной нервной систем и конечностей. В настоящее время эпидемиологическая картина распространенности и структуры врожденных пороков развития во всем мире выглядит стабильно.В статье проанализированы данные популяционного регистра врожденных пороков развития Московской области с 2011 по 2019 г., охват которого составил 83% рождений в регионе. Общая частота врожденных пороков развития составила 25,42 на 1000 рождений. Определено, что структура врожденных пороков развития в Московской области сопоставима с таковой, приведенной в международных исследованиях, однако следует обратить внимание на более низкую распространенность большинства групп пороков развития, что, скорее всего, связано с недостаточно полной регистрацией пороков. Регистр Московской области представляет актуальные и качественные данные по частоте и структуре врожденных пороков развития в регионе, что позволяет в случае необходимости экстраполировать полученные оценки и на другие субъекты Российской Федерации.

The aim of this work is to analyze the epidemiology of congenital malformations in different populations in comparison with the data of the regional register of congenital malformations of the Moscow Region. The article provides a brief description and results of the work of international organizations that collect and analyze data on the prevalence and structure of congenital anomalies — EUROCAT (European network of population-based registries for the epidemiological surveillance of congenital anomalies) and ICBDSR (The International Clearinghouse for Birth Defects Surveillance and Research). For countries where there are no monitoring systems for congenital malformations recording, the Modell World Database of Congenital Diseases (MGDb) has been developed, which makes it possible to calculate the estimated population prevalence of congenital malformations in these regions. According to international registers, the most common groups of congenital malformations are malformations of the cardiovascular, genitourinary, central nervous systems and malformations of the extremities. To date, the epidemiological picture of the prevalence and structure of congenital malformations around the world looks stable.The article analyzes data from the population-based register of congenital malformations in the Moscow Region from 2011 to 2019, with a coverage of 83% of births in the region. The overall prevalence of congenital malformations was 25.42 per 1000 births. It is determined that the structure of congenital malformations in the Moscow Region is comparable to the data of international studies, but one should pay attention to the lower frequency of most groups of malformations, which is most likely due to insufficient registration of malformations. The Moscow Region register of congenital malformations provides relevant and qualitative data on the frequency and structure of the congenital malformations in the region, which allows, if necessary, to extrapolate obtained prevalence rates to other constituent entities of the Russian Federation as well.

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