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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">genort</journal-id><journal-title-group><journal-title xml:lang="ru">Гений ортопедии</journal-title><trans-title-group xml:lang="en"><trans-title>Genij Ortopedii</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1028-4427</issn><issn pub-type="epub">2542-131X</issn><publisher><publisher-name>ЦЕНТР ИЛИЗАРОВА</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.18019/1028-4427-2021-27-4-461-467</article-id><article-id custom-type="elpub" pub-id-type="custom">genort-32</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>Оригинальные статьи</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>Original articles</subject></subj-group></article-categories><title-group><article-title>Несовершенный остеогенез: роль и место ортопедической хирургии нижних конечностей</article-title><trans-title-group xml:lang="en"><trans-title>Osteogenesis imperfecta: the role and place of orthopedic surgery of the lower extremities</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Попков</surname><given-names>Д. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Popkov</surname><given-names>D. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Попков Дмитрий Арнольдович, д. м. н., профессор РАН, членкорр. Французской Академии медицинских наук,</p><p>г. Курган</p></bio><bio xml:lang="en"><p>Dmitry A. Popkov, M.D., Ph.D., Professor of RAS, correspondent member French Academy of Medical Sciences,</p><p>Kurgan</p></bio><email xlink:type="simple">dpopkov@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мингазов</surname><given-names>Е. Р.</given-names></name><name name-style="western" xml:lang="en"><surname>Mingazov</surname><given-names>E. R.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Мингазов Эдуард Рифович,</p><p>г. Курган</p></bio><bio xml:lang="en"><p>Eduard R. Mingazov, M.D.,</p><p>Kurgan</p></bio><email xlink:type="simple">ed.dobryak@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Губин</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Gubin</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Губин Александр Вадимович, д. м. н.,</p><p>г. Москва</p></bio><bio xml:lang="en"><p>Alexander V. Gubin, M.D., Ph.D.,</p><p>Moscow</p></bio><email xlink:type="simple">alexander@gubin.spb.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гвоздев</surname><given-names>Н. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Gvozdev</surname><given-names>N. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Гвоздев Никита Сергеевич,</p><p>г. Курган</p></bio><bio xml:lang="en"><p>Nikita S. Gvozdev,</p><p>Kurgan</p></bio><email xlink:type="simple">gvozdev_n.s@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Аранович</surname><given-names>А. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Aranovich</surname><given-names>A. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Аранович Анна Майоровна, д. м. н., профессор,</p><p>г. Курган</p></bio><bio xml:lang="en"><p>Anna M. Aranovich, M.D., Ph.D., Professor,</p><p>Kurgan</p></bio><email xlink:type="simple">aranovich_anna@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Семенов</surname><given-names>А. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Semyonov</surname><given-names>A. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Семенов Александр Леонидович,</p><p>г. Новосибирск</p></bio><bio xml:lang="en"><p>Alexander L. Semyonov,</p><p>Novosibirsk</p></bio><email xlink:type="simple">semenov152@yandex.ru</email><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное учреждение «Национальный медицинский исследовательский центр травматологии и ортопедии имени академика Г.А. Илизарова» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Ilizarov National Medical Research Centre for Traumatology and Orthopedics</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное учреждение «Национальный медицинский исследовательский центр травматологии и ортопедии имени Н.Н. Приорова» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>National Medical Research Center of Traumatology and Orthopedics n.a. N.N. Priorov</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное учреждение «Новосибирский научно-исследовательский институт травматологии и ортопедии им. Я.Л. Цивьяна» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Novosibirsk Research Institute of Traumatology and Orthopaedics N.a. Ya.l. Tsivyan</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2021</year></pub-date><pub-date pub-type="epub"><day>28</day><month>08</month><year>2021</year></pub-date><volume>27</volume><issue>4</issue><issue-title>№ 4 (2021)</issue-title><fpage>461</fpage><lpage>467</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Попков Д.А., Мингазов Е.Р., Губин А.В., Гвоздев Н.С., Аранович А.М., Семенов А.Л., 2021</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="ru">Попков Д.А., Мингазов Е.Р., Губин А.В., Гвоздев Н.С., Аранович А.М., Семенов А.Л.</copyright-holder><copyright-holder xml:lang="en">Popkov D.A., Mingazov E.R., Gubin A.V., Gvozdev N.S., Aranovich A.M., Semyonov A.L.</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.ilizarov-journal.com/jour/article/view/32">https://www.ilizarov-journal.com/jour/article/view/32</self-uri><abstract><sec><title>Введение</title><p>Введение. Хирургическая коррекция ортопедических проблем у детей и взрослых с тяжелыми формами несовершенного остеогенеза вызывает интерес медицинского сообщества.</p><p>Целью данной статьи стал обзор современной релевантной литературы о роли ортопедической хирургии при лечении детей и взрослых с несовершенным остеогенезом и интеграции хирургии в мультидисциплинарный подход при лечении пациентов с несовершенным остеогенезом. Статья построена на материале доклада, созданного для впервые проводимой в России образовательной конференции European Pediatric Orthopaedic Society в Центре Илизарова в 2021 году.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. При подготовке обзора были использованы поисково-информационные источники: научный поисковый портал PubMed, Scopus, ResearchGate, РИНЦ, а также издательские продукты (Elsevier, Springer).</p></sec><sec><title>Результаты и обсуждение</title><p>Результаты и обсуждение. Публикация включает обсуждение классификации, показаний, особенностей хирургической техники и раннего послеоперационного периода, роли ранней функциональной нагрузки, особенностей отдаленных результатов лечения при использовании телескопических трансфизарных конструкций при коррекции деформаций длинных трубчатых костей нижних конечностей, а также аспектов сочетания ортопедической хирургии и бисфосфонат-терапии.</p></sec><sec><title>Заключение</title><p>Заключение. Ортопедическое сопровождение детей и взрослых с тяжелыми и средней степени тяжести формами несовершенного остеогенеза требует построения многофакторной стратегии, учитывающей требования физической терапии и медикаментозного лечения, а также применения специализированных имплантов, инструментария и способов хирургического вмешательства, где по-прежнему идет поиск оптимального решения. </p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. Surgical correction of orthopedic problems in children and adults with severe osteogenesis imperfecta is of interest to the medical community.</p><p>The aim of this study was to review the current relevant literature on the role of orthopedic surgery in the treatment of children and adults with osteogenesis imperfecta and the integration of surgery into a multidisciplinary approach to the treatment of patients with osteogenesis imperfecta. The review is based on the material of the report presented at the first educational conference of the European Pediatric Orthopedic Society which was held in Russia at the Ilizarov Center in 2021.</p></sec><sec><title>Material and methods</title><p>Material and methods. To prepare the review, we searched for information sources at the scientific platforms such as PubMed, Scopus, ResearchGate, RSCI, as well as published products (Elsevier, Springer).</p></sec><sec><title>Results and discussion</title><p>Results and discussion. The review includes a discussion of the classification, indications, features of the surgical technique and the early postoperative period, the role of early functional loading, the features of long-term treatment results of using telescopic transphyseal constructs in the correction of deformities of lower limb long bones as well as issues of the combination of orthopedic surgery and bisphosphonate therapy.</p></sec><sec><title>Conclusion</title><p>Conclusion. Orthopedic support of children and adults with severe and moderate types of osteogenesis imperfecta requires construction of a multifactorial strategy that takes into account the requirements of physical therapy and medication treatment, as well as the use of specialized implants, instrumentation and methods of surgical intervention, while an optimal solution has been still sought.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>несовершенный остеогенез</kwd><kwd>коррекция деформаций</kwd><kwd>телескопический остеосинтез</kwd></kwd-group><kwd-group xml:lang="en"><kwd>osteogenesis imperfecta</kwd><kwd>deformity correction</kwd><kwd>telescopic nail</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">Sillence D.O., SennA., Danks D.M. Genetic heterogeneity in osteogenesisimperfecta //J. Med. Genet. 1979. Vol. 16, No 2. P. 101-116. 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