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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">nodgo</journal-id><journal-title-group><journal-title xml:lang="ru">Российский журнал детской гематологии и онкологии (РЖДГиО)</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Journal of Pediatric Hematology and Oncology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2311-1267</issn><issn pub-type="epub">2413-5496</issn><publisher><publisher-name>LTD “Graphica”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21682/2311-1267-2022-9-1-29-44</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-800</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 ЛИТЕРАТУРЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>LITERATURE REVIEWS</subject></subj-group></article-categories><title-group><article-title>Спектр синдромов избыточного роста, связанных с мутацией PIK3CA. Обзор литературы</article-title><trans-title-group xml:lang="en"><trans-title>A spectrum of overgrowth syndromes associated with the PIK3CA mutation. Literature review</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-7846-3473</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сагоян</surname><given-names>Г. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Sagoyan</surname><given-names>G. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Гарик Барисович Сагоян, научный сотрудник, врач-детский онколог детского онкологического отделения хирургических методов лечения с проведением химиотерапии № 2 (опухолей торакоабдоминальной локализации) НИИ детской онкологии и гематологии, SPIN-код: 6304-0159</p><p>Москва</p></bio><bio xml:lang="en"><p>Researcher, Pediatric Oncologist of Pediatric Oncology Department of Surgical Methods of Treatment with Chemotherapy No. 2 (Tumors of Thoracoabdominal Localization) of Research Institute of Pediatric Oncology and Hematology, SPIN-code: 6304-0159</p><p>Moscow</p></bio><email xlink:type="simple">sagoyan-garik@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8552-7682</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Клецкая</surname><given-names>И. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Kletskaya</surname><given-names>I. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-патологоанатом патологоанатомического отделения</p><p>Москва</p></bio><bio xml:lang="en"><p>Pathologist Pathology Department at Russian Children’s Clinical Hospital</p><p>Moscow</p></bio><email xlink:type="simple">ikletskaya@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4529-7891</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Имянитов</surname><given-names>Е. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Imyanitov</surname><given-names>E. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>член-корр. РАН, д.м.н., профессор, руководитель научного отдела биологии опухолевого роста и лаборатории молекулярной онкологии, SPIN-код: 1909-7323</p><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Corresponding Member of RAS, Dr. of Sci. (Med.), Professor, Head of the Research Department of Biology of Tumor Growth and the Laboratory of Molecular Oncology, SPIN-code: 1909-7323</p><p>Saint Petersburg</p></bio><email xlink:type="simple">evgeny@imyanitov.spb.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0652-6049</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мареева</surname><given-names>Ю. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Mareeva</surname><given-names>Yu. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог консультативного отделения</p><p>Москва</p></bio><bio xml:lang="en"><p>Pediatric Oncologist of Advisory Department</p><p>Moscow</p></bio><email xlink:type="simple">7261610@mail.ru</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9054-5068</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Жуков</surname><given-names>Н. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Zhukov</surname><given-names>N. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., профессор, руководитель отдела мультидисциплинарной онкологии, SPIN-код: 8151-9305, ResearcherID: I-3833-2018</p><p>Москва</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Head of Multidisciplinary Oncology Department, ResearcherID: I-3833-2018, SPIN-code: 8151-9305</p><p>Moscow</p></bio><email xlink:type="simple">1cancerdoctor1@gmail.com</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7944-8438</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хагуров</surname><given-names>Р. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Khagurov</surname><given-names>R. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-детский хирург отделения микрохирургии</p><p>Москва</p></bio><bio xml:lang="en"><p>Surgeon of the Department of Reconstructive Microsurgery</p><p>Moscow</p></bio><email xlink:type="simple">dr.khagurov@gmail.com</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5489-1879</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сулейманова</surname><given-names>А. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Suleymanova</surname><given-names>A. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>научный сотрудник, врач-детский онколог детского онкологического отделения хирургических методов лечения с проведением химиотерапии № 2 (опухолей торакоабдоминальной локализации) НИИ детской онкологии и гематологии</p><p>Москва</p></bio><bio xml:lang="en"><p>Researcher, Pediatric Oncologist of Pediatric Oncology Department of Surgical Methods of Treatment with Chemotherapy No. 2 (Tumors of Thoracoabdominal Localization) of Research Institute of Pediatric Oncology and Hematology</p><p>Moscow</p></bio><email xlink:type="simple">aminasuleymanova313@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>НМИЦ онкологии им. Н.Н. Блохина</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>РДКБ РНИМУ им. Н.И. Пирогова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>НМИЦ онкологии им. Н.Н. Петрова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Petrov National Medical Research Center of Oncology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>НМИЦ ДГОИ им. Дмитрия Рогачева</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>ДГКБ им. Н.Ф. Филатова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.F. Filatov Children’s City Clinical Hospital, Moscow Department of Health</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2022</year></pub-date><pub-date pub-type="epub"><day>30</day><month>04</month><year>2022</year></pub-date><volume>9</volume><issue>1</issue><fpage>29</fpage><lpage>44</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Сагоян Г.Б., Клецкая И.С., Имянитов Е.Н., Мареева Ю.М., Жуков Н.В., Хагуров Р.А., Сулейманова А.М., 2022</copyright-statement><copyright-year>2022</copyright-year><copyright-holder xml:lang="ru">Сагоян Г.Б., Клецкая И.С., Имянитов Е.Н., Мареева Ю.М., Жуков Н.В., Хагуров Р.А., Сулейманова А.М.</copyright-holder><copyright-holder xml:lang="en">Sagoyan G.B., Kletskaya I.S., Imyanitov E.N., Mareeva Y.M., Zhukov N.V., Khagurov R.A., Suleymanova A.M.</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://journal.nodgo.org/jour/article/view/800">https://journal.nodgo.org/jour/article/view/800</self-uri><abstract><p>Спектр синдромов избыточного роста, связанных с мутацией PIK3CA (PIK3CA-Related Overgrowth Spectrum, PROS), – термин, объединяющий редкие заболевания, которые характеризуются пороками развития и чрезмерным разрастанием тканей. Эти нарушения вызваны соматическими мутациями в гене PIK3CA, произошедшими на этапе эмбриогенеза. В статье рассмотрены патогенез, клиническая картина, диагностика и лечение данного спектра синдромов.</p><p>В работе часть использованных иллюстраций заимствована, права авторов учтены и не нарушены. Заимствование разрешено журналами, где были опубликованы статьи.</p></abstract><trans-abstract xml:lang="en"><p>PIK3CA-Related Overgrowth Spectrum (PROS) refers to rare syndromes, which are characterized by malformations and excessive tissue growth and caused by somatic mutations in the PIK3CA gene occurring during embryogenesis. This article discusses the pathogenesis, clinical picture, diagnosis and treatment of these syndromes.</p><p>Some of the illustrations used in this article are copied from other resources, the rights of the authors are respected and not violated. The appropriate permissions from the journals have been obtained.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>синдром избыточного роста</kwd><kwd>PROS</kwd><kwd>дети</kwd><kwd>ингибиторы фосфатидилинозитола-3-киназы</kwd></kwd-group><kwd-group xml:lang="en"><kwd>overgrowth syndrome</kwd><kwd>PROS</kwd><kwd>children</kwd><kwd>phosphatidylinositol 3-kinase inhibitors</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">Parker V.E.R., Keppler-Noreuil K.M., Faivre L., Luu M., Oden N.L., De Silva L., Sapp J.C., Andrews K., Bardou M., Chen K.Y., Darling T.N., Gautier E., Goldspiel B.R., Hadj-Rabia S., Harris J., Kounidas G., Kumar P., Lindhurst M.J., Loﬀroy R., Martin L., Phan A., Rother K.I., Widemann B.C., Wolters P.L., Coubes Ch., Pinson L., Willems M., Vincent-Delorme C., PROMISE Working Group; Vabres P., Semple R.K., Biesecker L.G. 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