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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">medsovet</journal-id><journal-title-group><journal-title xml:lang="ru">Медицинский Совет</journal-title><trans-title-group xml:lang="en"><trans-title>Meditsinskiy sovet = Medical Council</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2079-701X</issn><issn pub-type="epub">2658-5790</issn><publisher><publisher-name>REMEDIUM GROUP Ltd.</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21518/ms2023-437</article-id><article-id custom-type="elpub" pub-id-type="custom">medsovet-7948</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>ПЕДИАТРИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>PEDIATRICS</subject></subj-group></article-categories><title-group><article-title>Особенности аритмического синдрома у детей на фоне недифференцированной дисплазии соединительной ткани</article-title><trans-title-group xml:lang="en"><trans-title>Features of arrhythmic syndrome in children with nonspecific connective tissue disorder</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2255-128X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Нечаева</surname><given-names>Г. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Nechaeva</surname><given-names>G. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Нечаева Галина Ивановна - д.м.н., профессор, профессор кафедры внутренних болезней и семейной медицины дополнительного профессионального образования.</p><p>644099, Омск, ул. Ленина, д. 12; profnechaeva@yandex.ru</p></bio><bio xml:lang="en"><p>Galina I. Nechaeva - Doc. Sci. (Med.), Professor, Professor of the Department of Internal Medicine and Family Medicine with the course Additional Professional Education.</p><p>12, Lenin St., Omsk, 644043</p></bio><email xlink:type="simple">profnechaeva@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/0000-0001-8390-343X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Дакуко</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Dakuko</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Дакуко Анастасия Николаевна - к.м.н., доцент кафедры госпитальной педиатрии с курсом дополнительного профессионального образования.</p><p>644099, Омск, ул. Ленина, д. 12</p></bio><bio xml:lang="en"><p>Anastasia N. Dakuko - Cand. Sci. (Med.), Associate Professor of the Department of Hospital Pediatrics with the course Additional Professional Education.</p><p>12, Lenin St., Omsk, 644043</p></bio><email xlink:type="simple">doc-man85@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/0000-0002-0601-7044</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Логинова</surname><given-names>Е. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Loginova</surname><given-names>E. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Логинова Екатерина Николаевна - к.м.н., доцент, доцент кафедры внутренних болезней и семейной медицины дополнительного профессионального образования.</p><p>644099, Омск, ул. Ленина, д. 12</p></bio><bio xml:lang="en"><p>Ekaterina N. Loginova - Candi. Sci. (Med.), Associate Professor, Associate Professor of the Department of Internal Medicine and Family Medicine with the course Additional Professional Education.</p><p>12, Lenin St., Omsk, 644043</p></bio><email xlink:type="simple">ekaterina.n.loginova@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/0000-0003-4016-2023</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Богатырев</surname><given-names>И. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Bogatyrev</surname><given-names>I. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Богатырев Илья Вячеславович, студент лечебного факультета.</p><p>644099, Омск, ул. Ленина, д. 12</p></bio><bio xml:lang="en"><p>Ilia V. Bogatyrev - Student of the Faculty of General Medicine.</p><p>12, Lenin St., Omsk, 644043</p></bio><email xlink:type="simple">ilyabogatyrev23339@icloud.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/0000-0002-3643-560X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шарун</surname><given-names>И. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Sharun</surname><given-names>I. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Шарун Иван Владимирович - старший преподаватель кафедры «Прикладная математика и фундаментальная информатика» факультета информационных технологий и компьютерных систем.</p><p>644050, Омск, проспект Мира, д. 11</p></bio><bio xml:lang="en"><p>Ivan V. Sharun - Senior Lecturer of the Department of Applied Mathematics and Fundamental Informatics, Faculty of Information Technologies and Computer Systems.</p><p>11, Рrospect Mira, Omsk, 644050</p></bio><email xlink:type="simple">ivansharun@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Омский государственный медицинский университет</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Omsk State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Омский государственный технический университет</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Omsk State Technical 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>21</day><month>01</month><year>2024</year></pub-date><volume>0</volume><issue>23</issue><fpage>294</fpage><lpage>302</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Нечаева Г.И., Дакуко А.Н., Логинова Е.Н., Богатырев И.В., Шарун И.В., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Нечаева Г.И., Дакуко А.Н., Логинова Е.Н., Богатырев И.В., Шарун И.В.</copyright-holder><copyright-holder xml:lang="en">Nechaeva G.I., Dakuko A.N., Loginova E.N., Bogatyrev I.V., Sharun I.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.med-sovet.pro/jour/article/view/7948">https://www.med-sovet.pro/jour/article/view/7948</self-uri><abstract><sec><title>Введение</title><p>Введение. При недифференцированной дисплазии соединительной ткани (НДСТ) наиболее значимым в плане прогноза для жизни является аритмический синдром. Поэтому важным остается внедрение в практику современных методов диагностики для предотвращения фатальных событий в детском и молодом возрасте.</p></sec><sec><title>Цель</title><p>Цель. Изучить особенности аритмического синдрома у детей с НДСТ во взаимосвязи с уровнем N-концевого промозгового натрийуретического пептида (NT-proBNP) и структурными изменениями сердца.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. В исследование включены 45 детей 10–17 лет с аритмическим синдромом на фоне НДСТ и 20 детей с нормальным ритмом сердца и единичными проявлениями НДСТ (группа контроля). Проведено изучение фенотипических маркеров НСДТ, уровня NT-proBNP, результатов мониторирования электрокардиограммы (ЭКГ) и данных speckle-tracking-эхокардиографии.</p></sec><sec><title>Результаты и обсуждение</title><p>Результаты и обсуждение. У детей с аритмическим синдромом на фоне НДСТ чаще встречались долихостеномелия, арахнодактилия, килевидная деформация грудной клетки II–III ст., сколиоз позвоночника II–III ст., гипермобильность суставов, голубые склеры. В структуре аритмического синдрома у детей с НДСТ преобладали гетеротопные нарушения сердечного ритма (желудочковая и наджелудочковая экстрасистолия, пароксизмальная наджелудочковая тахикардия). При проведении speckle-tracking-эхокардиографии выявлено раннее ремоделирование миокарда у детей с аритмией на фоне НДСТ в виде снижения продольной деформации по переднему базальному сегменту и глобальной продольной деформации миокарда левого желудочка. Установлена взаимосвязь между снижением локального продольного стрейна в переднем базальном сегменте и килевидной деформацией грудной клетки II–III ст., пролапсом митрального клапана, продольным плоскостопием, астенической формой грудной клетки, воронкообразной деформацией грудной клетки II–III ст. Выявлена ассоциация уровня NT-proBNP с диагностическим порогом НДСТ. При проведении корреляционного анализа у детей с аритмией на фоне НДСТ выявлена прямая средней степени зависимости статистически значимая взаимосвязь уровня NT-proBNP с частотой сердечных сокращений, наличием пролапса митрального клапана, нарушением сердечного ритма по типу желудочковой экстрасистолии.</p></sec><sec><title>Выводы</title><p>Выводы. Результаты исследования в будущем будут внедрены в разработку индивидуальных программ ведения пациентов с дисплазиями соединительной ткани. </p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. In case of nonspecific connective tissue disorder (NCTD), the most significant in terms of prognosis for life is arrhythmic syndrome. Therefore, it remains important to introduce modern diagnostic methods into practice to prevent fatal events in childhood and young people.</p></sec><sec><title>Aim</title><p>Aim. To study the features of arrhythmic syndrome in children with NCTD in relation to the level of N-terminal pro-brain natriuretic peptide (NT-proBNP) and structural changes in the heart.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. A total of 45 children aged 10–17 years with arrhythmic syndrome due to NCTD and 20 children with normal heart rhythm and isolated presentations of NCTD (control group) were enrolled in the study. NSDT phenotypic markers, NT-proBNP levels, electrocardiogram (ECG) monitoring findings and speckle-tracking echocardiography data were studied.</p></sec><sec><title>Results and discussion</title><p>Results and discussion. Children with arrhythmic syndrome and NCTD had more frequently dolichostenomelia, arachnodactyly, keeled chest deformity of II–III-degree, spinal scoliosis of II–III-degree, joint hypermobility, blue sclera. The structure of the arrhythmic syndrome in children with NCTD is – ventricular and supraventricular extrasystoles, paroxysmal supraventricular tachycardia. Speckle-tracking echocardiography showed a left ventricular strain in anterior basal segment and global longitudinal strain. There was association between a local longitudinal strain in the anterior basal segment and keeled chest deformity of II–III-degree, mitral valve prolapse, longitudinal flatfoot, asthenic shape of the chest, pectus excavatum deformity of II–III degree. There was association between the NT-proBNP level and the diagnostic threshold for NCTD. Patients with arrhythmic syndrome and NCTD had a direct, medium-degree significant correlation between the NT-proBNP level and heart rate, mitral valve prolapse and cardiac arrhythmia such as ventricular extrasystoles.</p></sec><sec><title>Conclusion</title><p>Conclusion. The results of the study will be implemented in the future in the individual programs for the management of patients with NCTD.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>дети</kwd><kwd>недифференцированная дисплазия соединительной ткани</kwd><kwd>нарушения сердечного ритма</kwd><kwd>диагностика</kwd><kwd>NT-proBNP</kwd></kwd-group><kwd-group xml:lang="en"><kwd>children</kwd><kwd>nonspecific connective tissue disorder</kwd><kwd>heart rhythm disturbances</kwd><kwd>diagnosis</kwd><kwd>NT-proBNP</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Исследование выполнено за счет гранта Российского научного фонда №22-25-20100 «Оценка структурно-функциональных изменений миокарда у пациентов с недифференцированной дисплазией соединительной ткани, проживающих в Омске и Омской области»</funding-statement><funding-statement xml:lang="en">The study was supported by the Russian Science Foundation grant No. 22-25-20100 “Assessment of structural and functional changes of the myocardium in patients with nonspecific connective tissue disorder living in Omsk and the Omsk region”</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">Аббакумова ЛН, Арсентьев ВГ, Кадурина ТИ, Копцева АВ, Краснова ЕЕ, Мамбетова АМ и др. 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