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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">vestar</journal-id><journal-title-group><journal-title xml:lang="ru">Вестник аритмологии</journal-title><trans-title-group xml:lang="en"><trans-title>Journal of Arrhythmology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1561-8641</issn><issn pub-type="epub">2658-7327</issn><publisher><publisher-name>НАО «Инкарт»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.35336/VA-1506</article-id><article-id custom-type="elpub" pub-id-type="custom">vestar-1565</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>КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CASE REPORTS</subject></subj-group></article-categories><title-group><article-title>Случай проспективного наблюдения ребенка с генетически подтвержденным синдромом кернса-сейра и имплантированным электрокардиостимулятором: клиническое наблюдение</article-title><trans-title-group xml:lang="en"><trans-title>Follow-up of a child with kearns-sayre syndrome and implanted pacemaker: a case report</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>Бокач</surname><given-names>А. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Bokach</surname><given-names>A. Y.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Караганда, ул. Крылова, д.23 </p></bio><bio xml:lang="en"><p>Karaganda, 23 Krylova str. </p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Игембеков</surname><given-names>Н. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Igembekov</surname><given-names>N. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Караганда, ул. Крылова, д.23 </p></bio><bio xml:lang="en"><p>Karaganda, 23 Krylova str. </p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Серикова</surname><given-names>Ж. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Serikova</surname><given-names>Zh. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Серикова Жания Ертаргиновна </p><p>Караганда, ул. Крылова, д.23 </p></bio><bio xml:lang="en"><p>Serikova Zhaniya </p><p>Karaganda, 23 Krylova str. </p></bio><email xlink:type="simple">m.zhaneka96@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru">КГП «Многопрофильная больница №2 города Караганды» управления здравоохранения Карагандинской области<country>Казахстан</country></aff><aff xml:lang="en">Multi-profile hospital No.2 in Karaganda<country>Kazakhstan</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2025</year></pub-date><pub-date pub-type="epub"><day>05</day><month>09</month><year>2025</year></pub-date><volume>32</volume><issue>3</issue><fpage>51</fpage><lpage>54</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Бокач А.Ю., Игембеков Н.С., Серикова Ж.Е., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Бокач А.Ю., Игембеков Н.С., Серикова Ж.Е.</copyright-holder><copyright-holder xml:lang="en">Bokach A.Y., Igembekov N.S., Serikova Z.E.</copyright-holder><license 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://vestar.elpub.ru/jour/article/view/1565">https://vestar.elpub.ru/jour/article/view/1565</self-uri><abstract><p>Представлен впервые зарегистрированный на территории Республики Казахстан клинический случай ребенка с редким митохондриальным заболеванием - синдромом Кернса-Сейра, у которого в связи с развитием полной атриовентрикулярной блокады был имплантирован электрокардиостимулятор. Обсуждаются вопросы диагностики и тактики ведения.</p></abstract><trans-abstract xml:lang="en"><p>A clinical case of a child with a rare mitochondrial disease, Kearns-Sayre syndrome, who had a pacemaker implanted due to the development of complete atrioventricular block, is presented for the first time in the Republic of Kazakhstan. The issues of complex diagnostics and management tactics are discussed.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>митохондриальная миопатия</kwd><kwd>выраженный неврологический дефицит</kwd><kwd>атриовентрикулярная блокада третьей степени</kwd><kwd>дети</kwd><kwd>имплантация электрокардиостимулятора у детей</kwd></kwd-group><kwd-group xml:lang="en"><kwd>mitochondrial myopathy</kwd><kwd>severe neurological deficit</kwd><kwd>atrioventricular block of the third degree in children</kwd><kwd>children</kwd><kwd>implantation of a pacemaker in children</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">Kearns TP, Sayre GP. Retinitis pigmentosa, external ophthalmophegia, and complete heart block: unusual syndrome with histologic study in one of two cases. AMA Arch Ophthalmol. 1958;60(2): 280-9.</mixed-citation><mixed-citation xml:lang="en">Kearns TP, Sayre GP. 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