nogrЭкспериментальная и клиническая гастроэнтерологияExperimental and Clinical Gastroenterology1682-8658«Global Media Technologies»nogr-199Research ArticleОБЗОРREVIEWЭПИДЕМИОЛОГИЧЕСКИЕ И КЛИНИКО-ГЕНЕТИЧЕСКИЕ ХАРАКТЕРИСТИКИ ПОРОКОВ РАЗВИТИЯ АНОРЕКТАЛЬНОГО ОТДЕЛАEPIDEMIOLOGICAL, CLINICAL AND GENETIC CHARACTERISTICS OF ANORECTAL MALFORMATIONS (REVIEW)ВыдрычЮ. В.VydrychYu. V.noemail@neicon.ruДемиковаН. С.DemikovaN. S.noemail@neicon.ruФилюшкинЮ. Н.FilyushkinYu. N.noemail@neicon.ruКалиненковаС. Г.KalinenkovaS. G.noemail@neicon.ruПодольнаяМ. А.PodolnayaM. A.noemail@neicon.ruЛапинаА. С.LapinaA. S.noemail@neicon.ruМашковА. Е.MashkovA. E.noemail@neicon.ruАсановА. Ю.AsanovA. Yu.aliy@rambler.ruГБОУ ВПО Первый МГМУ им. И. М. СеченоваРоссияI. M. Sechenov First Moscow State Medical UniversityRussian FederationОСП Научно-исследовательский клинический институт педиатрии ГБОУ ВПО РНИМУ им. Н. И. ПироговаРоссияPirogov Russian National Research Medical UniversityRussian FederationГБУЗ МО Московский Областной Научно-исследовательский клинический институт им. М. Ф. ВладимирскогоРоссияMoscow Regional Research Clinical Institute named after M. F. VladimirskyRussian Federation201620052016055864Copyright © Выдрыч Ю.В., Демикова Н.С., Филюшкин Ю.Н., Калиненкова С.Г., Подольная М.А., Лапина А.С., Машков А.Е., Асанов А.Ю., 20162016Выдрыч Ю.В., Демикова Н.С., Филюшкин Ю.Н., Калиненкова С.Г., Подольная М.А., Лапина А.С., Машков А.Е., Асанов А.Ю.Vydrych Y.V., Demikova N.S., Filyushkin Y.N., Kalinenkova S.G., Podolnaya M.A., Lapina A.S., Mashkov A.E., Asanov A.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.nogr.org/jour/article/view/199

В обзоре представлен анализ современных данных по эпидемиологии, этиологии и клинико-генетическим характеристикам врожденных пороков развития аноректального отдела пищеварительного тракта. По данным международных регистров врожденных пороков развития популяционная частота АРП варьирует от 2 до 5 на 10000 рождений. Не выявлено изменения частоты аноректальных аномалий во времени (отсутствие временных трендов). В большинстве исследований отмечается преобладание мужского пола среди больных с аноректальными пороками (АРП). Примерно 40 - 70% пациентов с АРП имеют врожденные пороки развития других органов и систем. Представлены данные об участии генетических и средовых факторов в происхождении АРП. Дана краткая клинико-генетическая характеристика наиболее частых синдромов (ассоциаций) с АРП.

The review presents an analysis of current data on the epidemiology, etiology, clinical and genetic characteristics of congenital anorectal malformations (AM). According to international registers of congenital malformations prevalence of the AM varies from 2 to 5 per 10,000 births. There was no change in the prevalence over time of AM (absence of temporal trends). Most studies have indicated the predominance of males among patients with AM. Approximately 40 - 70% of patients with AM have congenital malformations of other organs and systems. The data on the participation of genetic and environmental factors in the origin of the AM. A brief clinical and genetic characteristics of the most common syndromes (association) with the AM.

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