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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">patmedfar</journal-id><journal-title-group><journal-title xml:lang="ru">Пациентоориентированная медицина и фармация</journal-title><trans-title-group xml:lang="en"><trans-title>Patient-Oriented Medicine and Pharmacy</trans-title></trans-title-group></journal-title-group><issn pub-type="epub">2949-1924</issn><publisher><publisher-name>LLC Izdatelstvo OKI</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.37489/2949-1924-0023</article-id><article-id custom-type="elpub" pub-id-type="custom">patmedfar-42</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>ТРАВМАТОЛОГИЯ И ОРТОПЕДИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>TRAUMATOLOGY AND ORTHOPEDICS</subject></subj-group></article-categories><title-group><article-title>Отдалённый результат хирургических коррекций деформаций нижних конечностей с интрамедуллярным армированием на фоне остеомаляции у взрослого пациента с гипофосфатемическим рахитом (редкое клиническое наблюдение)</article-title><trans-title-group xml:lang="en"><trans-title>Long-term outcome of surgical correction of lower limb deformities with intramedullary reinforcement against the background of osteomalacia in an adult patient with hypophosphatemic rickets (rare clinical observation)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1135-4376</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Литвинов</surname><given-names>И. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Litvinov</surname><given-names>I. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Литвинов Игорь Иванович — д. м. н., доцент, зав. кафедрой травматологии, ортопедии и военно-полевой хирургии с курсом ИПДО</p><p>РИНЦ Author ID: 368753</p><p>Ярославль</p></bio><bio xml:lang="en"><p>Igor I. Litvinov — Doctor of Medical Sciences, Associate Professor, Head. Department of Traumatology, Orthopedics and Military Field Surgery with the EITI course</p><p>Author ID: 368753</p><p>Yaroslavl</p></bio><email xlink:type="simple">litorthorus@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>Бландинский</surname><given-names>В. Ф.</given-names></name><name name-style="western" xml:lang="en"><surname>Blandinskij</surname><given-names>V. F.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Бландинский Валерий Федорович — д. м. н., профессор, зав. кафедрой детской хирургии</p><p>РИНЦ Author ID: 911669</p><p>Ярославль</p></bio><bio xml:lang="en"><p>Valery F. Blandinskij — Doctor of Medical Sciences, Professor, Head. Department of Pediatric Surgery</p><p>Author ID: 911669</p><p>Yaroslavl</p></bio><email xlink:type="simple">oblandinskaya@yandex.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/0000-0001-7634-1280</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Савгачев</surname><given-names>В. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Savgachev</surname><given-names>V. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Савгачев Виталий Владимирович — к. м. н., доцент, кафедра травматологии, ортопедии и военно-полевой хирургии с курсом ИПДО</p><p>РИНЦ Author ID: 825495</p><p>Ярославль</p></bio><bio xml:lang="en"><p>Vitaly V. Savgachev — Candidate of Medical Sciences, Associate Professor, Department of Traumatology, Orthopedics and Military Field Surgery with the EITI course</p><p>Author ID: 825495</p><p>Yaroslavl</p></bio><email xlink:type="simple">hirurg2288@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБОУ ВО «Ярославский государственный медицинский университет» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Yaroslavl State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2023</year></pub-date><pub-date pub-type="epub"><day>07</day><month>09</month><year>2023</year></pub-date><volume>1</volume><issue>3</issue><fpage>41</fpage><lpage>51</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Литвинов И.И., Бландинский В.Ф., Савгачев В.В., 2023</copyright-statement><copyright-year>2023</copyright-year><copyright-holder xml:lang="ru">Литвинов И.И., Бландинский В.Ф., Савгачев В.В.</copyright-holder><copyright-holder xml:lang="en">Litvinov I.I., Blandinskij V.F., Savgachev V.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://www.pomph.ru/jour/article/view/42">https://www.pomph.ru/jour/article/view/42</self-uri><abstract><p>Актуальность. Рецидивы деформаций нижних конечностей после их оперативной коррекции у пациентов с гипофосфатемическим рахитом достигают 95%, что требует совершенствования подходов к лечению данной патологии. Целью исследования явилось изучение эффективности и безопасности этапного оперативного лечения, включающего остеотомии, последовательный чрескостный и внутрикостный блокирующий остеосинтез-армирование, у пациента с завершённым ростом, страдающего X-сцепленной доминантной формой гипофосфатемического рахита. Материалы и методы. Пациенту с завершённым ростом с X-сцепленной доминантной формой гипофосфатемического рахита в возрасте 21–28 лет были выполнены 11 операций на нижних конечностях по поводу их выраженных многоплоскостных мультиапикальных деформаций, неравенства длин сегментов на фоне остеомаляции. На бедренных костях произведены одномоментные коррекции с использованием остеотомий и внутрикостного блокирующего остеосинтеза-армирования. На костях голеней выполнены этапные коррекции с применением остеотомий, костной аутопластики, последовательного чрескостного и внутрикостного блокирующего остеосинтеза-армирования. Результаты. Неравенство длин бёдер и голеней устранено. Оси нижних конечностей скорректированы в норму. Рост пациента увеличился на 8 см. Он ходит до 25 тысяч шагов в день без средств дополнительной опоры. При наблюдении за пациентом в течение 8 лет рецидивов деформаций и других осложнений не отмечено. Вывод. Наш опыт свидетельствует о высокой эффективности и безопасности этапного хирургического лечения с использованием остеотомий, чрескостного и внутрикостного блокирующего остеосинтеза˗армирования для коррекции выраженных многоплоскостных мультиапикальных деформаций, неравенства длин костей нижних конечностей на фоне остеомаляции у пациента с завершённым ростом, страдающего X-сцепленной доминантной формой гипофосфатемического рахита.</p></abstract><trans-abstract xml:lang="en"><p>Relevance. Relapses of deformities of the lower extremities after their surgical correction in patients with hypophosphatemic rickets reach 95%, which requires improved approaches to the treatment of this pathology. The aim of the study was to study the efficacy and safety of staged surgical treatment, including osteotomy, sequential transosseous and intraosseous blocking osteosynthesis-reinforcement, in a patient with completed growth, suffering from X-linked dominant form of hypophosphatemic rickets. Materials and methods. A patient with completed growth with an X-linked dominant form of hypophosphatemic rickets at the age of 21–28 years underwent 11 operations on the lower extremities due to their pronounced multiplanar multiapical deformities, segment length inequality against the background of osteomalacia. Simultaneous corrections were made on the femurs using osteotomies and intraosseous blocking osteosynthesis-reinforcement. On the bones of the lower legs, staged corrections were performed using osteotomies, bone autoplasty, sequential transosseous and intraosseous blocking osteosynthesis-reinforcement. Results. The inequality of the lengths of the femurs and lower legs is eliminated. The axes of the lower extremities were corrected to normal. The patient's height has increased by 8 cm. He walks up to 25,000 steps a day without additional support. When observing the patient for 8 years, no relapses of deformities and other complications were noted. Conclusion. Our experience testifies to the high efficiency and safety of staged surgical treatment using osteotomies, transosseous and intraosseous blocking osteosynthesis-reinforcement for the correction of pronounced multiplanar multiapical deformities, inequality in the length of the bones of the lower extremities against the background of osteomalacia in the patient with completed growth, suffering from an X-linked dominant form of hypophosphatemic rickets.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>гипофосфатемический рахит</kwd><kwd>коррекция деформаций</kwd><kwd>интрамедуллярное армирование</kwd><kwd>генетическое заболевание</kwd><kwd>редкое заболевание</kwd></kwd-group><kwd-group xml:lang="en"><kwd>hypophosphatemic rickets</kwd><kwd>deformity correction</kwd><kwd>intramedullary reinforcement</kwd><kwd>genetic disease</kwd><kwd>rare disease</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">Баранов А.А., Намазова-Баранова Л.С., Цыгин А.Н. и др. 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