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<article article-type="review-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">fcmedicine</journal-id><journal-title-group><journal-title xml:lang="ru">Фундаментальная и клиническая медицина</journal-title><trans-title-group xml:lang="en"><trans-title>Fundamental and Clinical Medicine</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2500-0764</issn><issn pub-type="epub">2542-0941</issn><publisher><publisher-name>КемГМУ</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.23946/2500-0764-2025-10-4-22-31</article-id><article-id custom-type="elpub" pub-id-type="custom">fcmedicine-1103</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>ПАТОЛОГИЧЕСКАЯ ФИЗИОЛОГИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>PATHOPHYSIOLOGY</subject></subj-group></article-categories><title-group><article-title>Патогенез острого надпочечникового криза у детей: современные представления и новые патогенетические механизмы</article-title><trans-title-group xml:lang="en"><trans-title>Pathogenesis of acute adrenal crisis in children: current concepts and emerging mechanisms</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4705-3823</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Быков</surname><given-names>Ю. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Bykov</surname><given-names>Yu. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Быков Юрий Витальевич, кандидат медицинских наук, доцент кафедры анестезиологии и реаниматологи с курсом дополнительного профессионального образования</p><p>ул. Мира, д. 310, г. Ставрополь, 355020, Россия</p></bio><bio xml:lang="en"><p>Dr. Yuri V. Bykov, MD, Cand. Sci. (Medicine), Associate Professor of the Department of Anesthesiology and Intensive Care Medicine with a Course of Additional Professional Education</p><p>Mira Street, 310, Stavropol, 355020, Russia</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Ставропольский государственный медицинский университет</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Stavropol State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2025</year></pub-date><pub-date pub-type="epub"><day>24</day><month>12</month><year>2025</year></pub-date><volume>10</volume><issue>4</issue><fpage>22</fpage><lpage>31</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Быков Ю.В., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Быков Ю.В.</copyright-holder><copyright-holder xml:lang="en">Bykov Y.V.</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://fcm.kemsmu.ru/jour/article/view/1103">https://fcm.kemsmu.ru/jour/article/view/1103</self-uri><abstract><p>Цель. Обзор патогенетических механизмов, лежащих в основе развития острого надпочечникового криза (ОНК) у детей, с акцентом на современные экспериментальные и клинические данные. Материалы и методы. Проведен поиск в базах данных MEDLINE, Embase, Scopus, Cochrane, PubMed, Google Scholar, Elibrary, а также в прямых и обратных ссылках на исследования, опубликованные в 2019–2025 гг. на русском и английском языках с последующим анализом 40 отечественных и зарубежных публикаций, включающих результаты клинических, экспериментальных и геномных исследований, а также систематические обзоры и метаанализы, посвящённые патогенезу надпочечниковой недостаточности и адреналового криза в педиатрии. Результаты. Классический патогенез ОНК связывается с дефицитом кортизола и альдостерона, приводящим к тяжёлым метаболическим и гемодинамическим нарушениям. Однако в последние годы сформировалось новое представление о мультифакторной природе синдрома, включающего системные, молекулярные, иммунные и микробиотные компоненты. Проанализированы ключевые этиологические формы, включая врождённую гиперплазию коры надпочечников, глюкокортикоид-индуцированную недостаточность и аутоиммунные формы заболевания. Представлены данные о вовлечении провоспалительных цитокинов (ИЛ-6, TNF-α), митохондриальной дисфункции, нарушений экспрессии рецепторов АКТГ (MC2R, MRAP), а также эпигенетических модификаций (NR0B1, CYP21A2). Особое внимание уделено роли сепсис-индуцированной недостаточности, эндотелиальной дисфункции и влиянию оси «микробиота–надпочечники» на развитие криза. Заключение. Современное понимание патогенеза ОНК выходит за рамки классической гормональной модели и включает сложные взаимодействия воспалительных, митохондриальных, иммунных, генетических и микробиотных факторов. Обоснована необходимость ранней диагностики, молекулярной стратификации пациентов и персонализированного подхода к терапии. Эти данные требуют пересмотра стратегий профилактики и лечения в педиатрической практике, с ориентацией на таргетные и междисциплинарные методы вмешательства.</p></abstract><trans-abstract xml:lang="en"><p>Aim. The aim of this article is to provide a comprehensive review of the pathogenetic mechanisms underlying the development of acute adrenal crisis (AAC) in children, with an emphasis on recent experimental and clinical findings. Materials and Methods. A comprehensive literature search was conducted using MEDLINE, Embase, Scopus, Cochrane, PubMed, Google Scholar and Elibrary databases, as well as through citation tracking of studies published between 2019 and June 2025. A total of 40 national and international publications were analyzed, including clinical, experimental, and genomic studies, as well as systematic reviews and meta-analyses focused on the pathogenesis of adrenal insufficiency and adrenal crisis in pediatric populations. Results. The classical pathogenesis of AAC is associated with cortisol and aldosterone deficiency, leading to severe metabolic and hemodynamic disturbances. However, in recent years, a new concept of the multifactorial nature of this syndrome has emerged, encompassing systemic, molecular, immune, and microbiota-related components. Key etiological forms have been analyzed, including congenital adrenal hyperplasia, glucocorticoid-induced adrenal insufficiency, and autoimmune adrenalitis. Data are presented on the involvement of pro-inflammatory cytokines (IL-6, TNF-α), mitochondrial dysfunction, altered expression of ACTH receptors (MC2R, MRAP), and epigenetic modifications (NR0B1, CYP21A2). Particular attention is given to sepsis-induced adrenal dysfunction, endothelial impairment, and the influence of the gut–adrenal axis on crisis development. Conclusion. The current understanding of AAC pathogenesis extends beyond the classical hormonal deficiency model and includes complex interactions among inflammatory, mitochondrial, immune, genetic, and microbiota-related mechanisms. The importance of early diagnosis, molecular stratification, and personalized treatment strategies is emphasized. These findings highlight the need to revise current approaches to prevention and management in pediatric practice, with a focus on targeted and multidisciplinary therapeutic interventions.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>острый надпочечниковый криз</kwd><kwd>надпочечниковая недостаточность</kwd><kwd>дети</kwd><kwd>патогенез</kwd><kwd>цитокины</kwd><kwd>митохондриальная дисфункция</kwd></kwd-group><kwd-group xml:lang="en"><kwd>acute adrenal crisis</kwd><kwd>adrenal insufficiency</kwd><kwd>children</kwd><kwd>pathogenesis</kwd><kwd>cytokines</kwd><kwd>mitochondrial dysfunction</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">Improda N., Chioma L., Capalbo D., Salerno M., Di Mase R., De Angelis S., et al. Glucocorticoid treatment and adrenal suppression in children: current view and open issues. J. Endocrinol. 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