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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">rosped</journal-id><journal-title-group><journal-title xml:lang="ru">Российский педиатрический журнал имени М.Я. Студеникина</journal-title><trans-title-group xml:lang="en"><trans-title>M.Ya. Studenikin Russian Pediatric Journal</trans-title></trans-title-group></journal-title-group><publisher><publisher-name>ФГАУ «НМИЦ здоровья детей» Минздрава России</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.46563/1560-9561-2024-27-1-66-70</article-id><article-id custom-type="edn" pub-id-type="custom">ewwuxu</article-id><article-id custom-type="elpub" pub-id-type="custom">rosped-536</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>ОБЗОРЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEWS</subject></subj-group></article-categories><title-group><article-title>Заболевания и синдромы, сочетающиеся с односторонней агенезией почки у детей</article-title><trans-title-group xml:lang="en"><trans-title>Diseases and syndromes associated with unilateral renal agenesis in children</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0009-6120-7009</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ромашова</surname><given-names>Анастасия Александровна</given-names></name><name name-style="western" xml:lang="en"><surname>Romashova</surname><given-names>Anastasia A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Клинический ординатор по специальности «детская урология-андрология» ФГАУ «НМИЦ здоровья детей» Минздрава России</p><p>e-mail: anastasi_romashova@mail.ru</p></bio><bio xml:lang="en"><p>Clinical Resident in Pediatric Urology Andrology Department, National Medical Research Center for Children’s Health, Moscow, Moscow, 119991, Russian Federation</p><p>e-mail: anastasi_romashova@mail.ru</p></bio><email xlink:type="simple">anastasi_romashova@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГАУ «Национальный медицинский исследовательский центр здоровья детей» Минздрава России</institution></aff><aff xml:lang="en"><institution>National Medical Research Center for Children’s Health</institution></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>04</day><month>04</month><year>2024</year></pub-date><volume>27</volume><issue>1</issue><fpage>66</fpage><lpage>70</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Ромашова А.А., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Ромашова А.А.</copyright-holder><copyright-holder xml:lang="en">Romashova A.A.</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.rosped.ru/jour/article/view/536">https://www.rosped.ru/jour/article/view/536</self-uri><abstract><p>Частота врождённых пороков развития ежегодно увеличивается, при этом в структуре пороков развития мочевой системы нарушения развития одной почки составляют 4–8%. При условии нормального развития контралатерального органа агенезия почки (АгП) клинически не проявляется и обычно выявляется во время профилактического осмотра ребёнка или обследования по поводу сочетанных аномалий развития половой и мочевыделительной систем. АгП формируется при нарушениях межтканевого взаимодействия между зачатком мочеточника и метанефрогенной тканью с 4-й по 8-ю неделю гестации. К этому моменту мезонефральные (вольфовы) протоки уже полностью сформированы, в отличие от парамезонефральных (мюллеровых) протоков, которые развиваются только к 5-й неделе внутриутробного периода, т. е. в период высокого риска возникновения пороков развития мочевыделительной системы. Соответственно аномалии женской половой системы встречаются чаще, чем мужской, с сопутствующей агенезией ипсилатеральной почки. Диагностирование односторонней АгП возможно при выполнении антенатального скрининга или профилактических осмотров в декретированные сроки. У девочек распространёнными аномалиями половых органов являются истинная однорогая матка (65%), двурогая матка с одним рудиментарным рогом (7,3%), атрофия одной маточной трубы и яичника и др. У мальчиков при односторонней АгП семенные пузырьки, предстательная железа и придаток яичка могут быть рудиментарными или отсутствовать.</p><p>АгП является составляющей таких генетических синдромов, как синдром OHVIRA, Каллмана, Циннера, Майера–Рокитанского–Кюстера–Хаузера. АгП сочетается с другими врождёнными аномалиями почек и внепочечными аномалиями, включая пороки развития желудочно-кишечного тракта, сердца и скелетно-мышечной системы. Следовательно, дети с АгП должны быть комплексно обследованы для исключения сопутствующих пороков и аномалий развития.</p><sec><title>Финансирование</title><p>Финансирование. Исследование не имело финансовой поддержки.</p></sec><sec><title>Конфликт интересов</title><p>Конфликт интересов. Автор подтверждает отсутствие конфликта интересов.</p></sec><sec><title>Поступила 26</title><p>Поступила 26.12.2023Принята к печати 30.01.2024Опубликована 28.02.2024</p></sec></abstract><trans-abstract xml:lang="en"><p>The incidence of congenital malformations is increasing annually, with a single kidney developmental disorder accounting for 4–8% of urinary system malformations. If the contralateral organ is normal, agenesis of the kidney is not clinically apparent and is usually detected during a preventive examination of the child or during an examination for combined anomalies of the genitourinary and urinary system.This pathology occurs due to a disruption of the interstitial interaction between the ureteral bud and the metanephrogenic tissue from  4th to 8th gestation weeks. By this time, the mesonephral (Wolff) ducts are already fully formed, in contrast to the paramesonephral (Müllerian) ducts, which develop only by the 5th week of the intrauterine period, that is, in the period of high risk of malformations of the urinary system. Accordingly, anomalies of the female genital system are more common than those of the male one, with concomitant agenesis of the ipsilateral kidney. Diagnosis of unilateral agenesis of the kidney is possible when performing routine antenatal screening or conducting preventive examinations at decreed dates. In girls, the most common genital anomalies are true unicornuate uterus (65%), bicornuate uterus with one rudimentary horn (7.3%), atrophy of one fallopian tube and ovary, absence or hypoplasia of the vagina, and vaginal doubling. In boys with unilateral agenesis of the kidney, the seminal vesicles, prostate gland, and testicular appendage may be rudimentary or absent. Agenesis of the kidney is a component of such genetic syndromes as OHVIRA, Kallman, Zinner, and Mayer–Rokitansky–Küster–Hauser syndromes. It is also combined with other congenital anomalies of the kidney and urinary tract and extrarenal anomalies, including mainly malformations of the gastrointestinal tract, heart and musculoskeletal system. Consequently, children with this pathology should be fully screened to rule out associated malformations and anomalies.</p><sec><title>Acknowledgement</title><p>Acknowledgement. The study had no financial support.</p></sec><sec><title>Conflict of interest</title><p>Conflict of interest. The author has no conflicts of interest to declare.</p></sec><sec><title>Received</title><p>Received: December 26, 2023Accepted: January 30, 2024Published: February 28, 2024</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>обзор</kwd><kwd>агенезия почки</kwd><kwd>генитальные аномалии</kwd><kwd>эмбриология почек</kwd></kwd-group><kwd-group xml:lang="en"><kwd>literature review</kwd><kwd>renal agenesis</kwd><kwd>genital anomalies</kwd><kwd>renal embryology</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">Jelin A. Renal agenesis. Am. J. Obstet. Gynecol. 2021; 225(5): B28–30. https://doi.org/10.1016/j.ajog.2021.06.048</mixed-citation><mixed-citation xml:lang="en">Jelin A. Renal agenesis. Am. J. Obstet. 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