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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">vsp</journal-id><journal-title-group><journal-title xml:lang="ru">Вопросы современной педиатрии</journal-title><trans-title-group xml:lang="en"><trans-title>Current Pediatrics</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1682-5527</issn><issn pub-type="epub">1682-5535</issn><publisher><publisher-name>Издательство «ПедиатрЪ»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.15690/vsp.v23i3.2768</article-id><article-id custom-type="elpub" pub-id-type="custom">vsp-3519</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>ОБЗОР ЛИТЕРАТУРЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEW</subject></subj-group></article-categories><title-group><article-title>Современные представления о воспалительных заболеваниях кишечника у детей с очень ранним и ранним началом</article-title><trans-title-group xml:lang="en"><trans-title>Modern View on Very Early Onset and Early Onset Inflammatory Bowel Diseases 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/0000-0001-7308-7280</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хавкин</surname><given-names>А. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Khavkin</surname><given-names>Anatoly I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Хавкин Анатолий Ильич - доктор медицинских наук, профессор, заведующий кафедрой гастроэнтерологии и диетологии им. А.В. Мазурина, руководитель Московского областного центра детской гастроэнтерологии и гепатологии НИКИ детства Минздрава Московской области, профессор кафедры педиатрии с курсом детских хирургических болезней МИ ФГАОУ ВО НИУ «БелГУ» МР.</p><p>115093, Москва, ул. Большая Серпуховская, 62, тел.: +7 (499) 237-02-23</p></bio><bio xml:lang="en"><p>Mytishchi, Belgorod</p></bio><email xlink:type="simple">gastropedclin@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0002-7103-6468</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Пермякова</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Permyakova</surname><given-names>Anastasiya A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Saint Petersburg</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6640-2155</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Цепилова</surname><given-names>М. О.</given-names></name><name name-style="western" xml:lang="en"><surname>Tsepilova</surname><given-names>Mariya O.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Saint Petersburg</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6939-6961</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Каплина</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Kaplina</surname><given-names>Aleksandra V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Saint Petersburg</p></bio><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0331-0963</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ситкин</surname><given-names>С. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Sitkin</surname><given-names>Stanislav I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Saint Petersburg</p></bio><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3697-4283</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сурков</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Surkov</surname><given-names>Andrey N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><bio xml:lang="en"><p>Moscow</p></bio><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0004-1751-1293</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гетманов</surname><given-names>С. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Getmanov</surname><given-names>Stanislav D.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><bio xml:lang="en"><p>Moscow</p></bio><xref ref-type="aff" rid="aff-6"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>НИКИ детства; НИУ «БелГУ»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research and Clinical Institute for Children; Belgorod State University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>СПбГПМУ</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Saint Petersburg State Pediatric Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>НМИЦ им. В.А. Алмазова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Almazov National Medical Research Centre</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>НМИЦ им. В.А. Алмазова; СЗГМУ им. И.И. Мечникова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Almazov National Medical Research Centre; North-Western State Medical University named after I.I. Mechnikov</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>НИИ педиатрии и охраны здоровья детей НКЦ №2 ФГБНУ «РНЦХ им. акад. Б.В. Петровского»; РНИМУ им. Н.И. Пирогова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-6"><aff xml:lang="ru"><institution>РНИМУ им. Н.И. Пирогова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Pirogov Russian National Research Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>12</day><month>07</month><year>2024</year></pub-date><volume>23</volume><issue>3</issue><fpage>145</fpage><lpage>151</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Хавкин А.И., Пермякова А.А., Цепилова М.О., Каплина А.В., Ситкин С.И., Сурков А.Н., Гетманов С.Д., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Хавкин А.И., Пермякова А.А., Цепилова М.О., Каплина А.В., Ситкин С.И., Сурков А.Н., Гетманов С.Д.</copyright-holder><copyright-holder xml:lang="en">Khavkin A.I., Permyakova A.A., Tsepilova M.O., Kaplina A.V., Sitkin S.I., Surkov A.N., Getmanov S.D.</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://vsp.spr-journal.ru/jour/article/view/3519">https://vsp.spr-journal.ru/jour/article/view/3519</self-uri><abstract><p>На сегодняшний день актуальной проблемой детской гастроэнтерологии является изучение воспалительных заболеваний кишечника с очень ранним началом (ВЗК-ОРН), которые имеют уникальные генетические, клинические, иммунологические, морфологические, лабораторные признаки. Считается, что ВЗК-ОРН зачастую является моногенным заболеванием, особенно в сочетании с врожденными дефектами иммунитета, что приводит к трудностям диагностики и лечения этой патологии. Несмотря на это, систематизация сведений о данной группе нозологических форм ВЗК практически не проводится. В статье представлен обзор имеющейся информации об этиологических факторах, вариантах течения и возможностях терапии ВЗК-ОРН.</p></abstract><trans-abstract xml:lang="en"><p>Nowadays, an urgent problem of pediatric gastroenterology is the study of inflammatory bowel diseases with very early onset (VEO-IBD), which have unique genetic, clinical, immunological, morphological, and laboratory sings. Early VEO-IBD is usually considered as monogenic disease, especially in combination with congenital immune defects, which leads to difficulties in diagnosis and management this pathology. Despite this, systematization of information about this group of nosological forms of IBD is practically not carried out. This article presents a review of the available information on etiological factors, course variants, and therapeutic options for VEO-IBD.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>воспалительные заболевания кишечника с ранним началом</kwd><kwd>воспалительные заболевания кишечника с очень ранним началом</kwd><kwd>болезнь Крона</kwd><kwd>язвенный колит</kwd><kwd>недифференцированный колит</kwd><kwd>некротизирующий энтероколит</kwd></kwd-group><kwd-group xml:lang="en"><kwd>early onset inflammatory bowel disease</kwd><kwd>very early onset inflammatory bowel disease</kwd><kwd>Crohn's disease</kwd><kwd>ulcerative colitis</kwd><kwd>indeterminate colitis</kwd><kwd>necrotizing enterocolitis</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Отсутствует</funding-statement><funding-statement xml:lang="en">Not declared</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Ouahed J, Spencer E, Kotlarz D, et al. 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