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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">persmed</journal-id><journal-title-group><journal-title xml:lang="ru">Российский журнал персонализированной медицины</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Journal for Personalized Medicine</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2782-3806</issn><issn pub-type="epub">2782-3814</issn><publisher><publisher-name>ФОНД АЛМАЗОВА</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.18705/2782-3806-2024-4-6-464-476</article-id><article-id custom-type="edn" pub-id-type="custom">GJAFJE</article-id><article-id custom-type="elpub" pub-id-type="custom">persmed-293</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>КАРДИОЛОГИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CARDIOLOGY</subject></subj-group></article-categories><title-group><article-title>Регистр как инструмент оценки экономических затрат на ведение пациентов детского возраста с гипертрофической кардиомиопатией</article-title><trans-title-group xml:lang="en"><trans-title>Registry as a tool for assessing economic costs of managing pediatric patients with hypertrophic cardiomyopathy</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>Алексеева</surname><given-names>Д. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Alekseeva</surname><given-names>D. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Алексеева Дарья Юрьевна, к.м.н., научный сотрудник НИО неизвестных, редких и генетически обусловленных заболеваний НЦМУ «Центр персонализированной медицины»</p><p>ул. Аккуратова, д. 2, Санкт-Петербург, 197341</p></bio><bio xml:lang="en"><p>Alekseeva Darya Yu., СM, Researcher, Research Institute of Unknown, Rare and Genetically Caused Diseases, World-Class Research Centre for Personalized Medicine</p><p>Akkuratova str., 2, Saint Petersburg, 197341</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>Фетисова</surname><given-names>С. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Fetisova</surname><given-names>S. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Фетисова Светлана Григорьевна, младший научный сотрудник НИЦ неизвестных, редких и генетически обусловленных заболеваний НЦМУ «Центр персонализированной медицины», врач — детский кардиолог отделения кардиологии и медицинской реабилитации Детского лечебно-реабилитационного комплекса Института перинатологии и педиатрии</p><p>ул. Аккуратова, д. 2, Санкт-Петербург, 197341</p></bio><bio xml:lang="en"><p>Fetisova Svetlana G., Junior Researcher, Research Institute of Unknown, Rare and Genetically Caused Diseases, World-Class Research Centre for Personalized Medicine; Pediatric Cardiologist, Department of Pediatric Cardiology and Medical Rehabilitation of Perinatology and Pediatrics</p><p>Akkuratova str., 2, Saint Petersburg, 197341</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>Первунина</surname><given-names>Т. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Pervunina</surname><given-names>T. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Первунина Татьяна Михайловна, д.м.н., директор Института перинатологии и педиатрии, заведующий кафедрой перинатологии и педиатрии</p><p>ул. Аккуратова, д. 2, Санкт-Петербург, 197341</p></bio><bio xml:lang="en"><p>Pervunina Tatyana M., MD, pediatrician, Director of the Institute of Perinatology and Pediatrics</p><p>Akkuratova str., 2, Saint Petersburg, 197341</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>Конради</surname><given-names>А. О.</given-names></name><name name-style="western" xml:lang="en"><surname>Konradi</surname><given-names>A. O.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Конради Александра Олеговна, д.м.н., профессор, академик РАН, заместитель генерального директора по научной работе, заведующий НИО артериальной гипертензии, заведующий кафедрой организации управления и экономики здравоохранения Института медицинского образования</p><p>ул. Аккуратова, д. 2, Санкт-Петербург, 197341</p></bio><bio xml:lang="en"><p>Konradi Alexandra O., PhD, MD, Professor, Academician of the Russian Academy of Sciences, Deputy Director General for Research; Head of the Research Institute of Arterial Hypertension, Head of the Department of Healthcare Management and Economics at the Institute of Medical Education</p><p>Akkuratova str., 2, Saint Petersburg, 197341</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>Вершинина</surname><given-names>Т. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Vershinina</surname><given-names>T. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Вершинина Татьяна Леонидовна, детский кардиолог высшей категории, заведующий отделением детской кардиологии и медицинской реабилитации Детского лечебно-реабилитационного комплекса</p><p>ул. Аккуратова, д. 2, Санкт-Петербург, 197341</p></bio><bio xml:lang="en"><p>Vershinina Tatyana L., pediatric cardiologist of the highest category, Head of the Department of Pediatric Cardiology and Medical Rehabilitation of Perinatology and Pediatrics</p><p>Akkuratova str., 2, Saint Petersburg, 197341</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>Ситникова</surname><given-names>К. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Sitnikova</surname><given-names>K. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Ситникова Ксения Алексеевна, клинический ординатор по специальности «Детская кардиология» </p><p>ул. Аккуратова, д. 2, Санкт-Петербург, 197341</p></bio><bio xml:lang="en"><p>Sitnikova Ksenia A., clinical resident in the specialty Pediatric Cardiology of the Medical Faculty of the Institute of Medical Education</p><p>Akkuratova str., 2, Saint Petersburg, 197341</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>Васичкина</surname><given-names>Е. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Vasichkina</surname><given-names>E. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Васичкина Елена Сергеевна, д.м.н., руководитель НИЦ неизвестных, редких и генетически обусловленных заболеваний НЦМУ «Центр персонализированной медицины», профессор кафедры перинатологии и педиатрии лечебного факультета Института медицинского образования</p><p>ул. Аккуратова, д. 2, Санкт-Петербург, 197341</p></bio><bio xml:lang="en"><p>Vasichkina Elena S., PhD, MD, Head of the Scientific Research Centre for Unknown, Rare and Genetically Caused Diseases of the World-Class Research Centre for Personalized Medicine; Professor of the Department of Pediatric Diseases of the Medical Faculty of the Institute of Medical Education</p><p>Akkuratova str., 2, Saint Petersburg, 197341</p></bio><email xlink:type="simple">vasichkinalena@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное учреждение «Национальный медицинский исследовательский центр имени В. А. Алмазова» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Almazov National Medical Research Centre</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>22</day><month>12</month><year>2024</year></pub-date><volume>4</volume><issue>6</issue><fpage>464</fpage><lpage>476</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Алексеева Д.Ю., Фетисова С.Г., Первунина Т.М., Конради А.О., Вершинина Т.Л., Ситникова К.А., Васичкина Е.С., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Алексеева Д.Ю., Фетисова С.Г., Первунина Т.М., Конради А.О., Вершинина Т.Л., Ситникова К.А., Васичкина Е.С.</copyright-holder><copyright-holder xml:lang="en">Alekseeva D.Y., Fetisova S.G., Pervunina T.M., Konradi A.O., Vershinina T.L., Sitnikova K.A., Vasichkina E.S.</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://persmed.elpub.ru/jour/article/view/293">https://persmed.elpub.ru/jour/article/view/293</self-uri><abstract><p>Актуальность. Гипертрофическая кардиомиопатия (ГКМП) является одной из самых распространенных кардиомиопатий в педиатрической популяции: 2,9 на 100 000 детей. Ведение пациентов с ГКМП требует высоких затрат здравоохранения. Исследования на основе данных регистра играют все большую роль в принятии решений в области здравоохранения. Цель исследования: анализ экономических затрат со стороны государства на ведение пациентов детского возраста с ГКМП по данным регистра пациентов детского возраста. Материалы и методы. Исследование выполнено на базе отделения детской кардиологии и медицинской реабилитации ФГБУ «НМИЦ им. В. А. Алмазова» Минздрава России. В настоящий момент в электронный регистр ГКМП у детей внесены данные о 159 детях, среди них 52 (34 %) девочки. Средний возраст дебюта заболевания составил 5 лет [0; 11], также 68 (44,4 %) детей имели дебют гипертрофии миокарда в возрасте до 1 года жизни. Были рассчитаны медицинские затраты, которые включали прямые затраты на лекарственную терапию, хирургические методы лечения (имплантация кардиовертера-дефибриллятора и миоэктомия) и госпитализацию, а также непрямые затраты — стоимость родственного ухода; выплаты пенсий по инвалидности, ущерб по показателю внутреннего валового продукта (ВВП), возникающий в связи со смертью пациентов в течение периода наблюдения. Результаты. Общая величина экономических затрат в течение года, обусловленных ГКМП, с позиции государства составила 107,8 млн руб., из них прямые медицинские затраты — 27,96 млн руб., прямые немедицинские затраты — 13,17 млн руб. и косвенные (непрямые) затраты — 66,7 млн руб. Основная доля экономического бремени (61,8 %) была обусловлена косвенными затратами. Прямые медицинские затраты составили только 26 % от всех экономических затрат. Средние затраты на 1 пациента на родственный уход составили 11 263,89 руб., а их общая величина на всю группу пациентов с ГКМП была равна 1,04 млн руб. Заключение. Полученные в нашем исследовании промежуточные результаты позволяют оценить экономические затраты со стороны государства на ведение пациентов с ГКМП. Оптимизация использования ресурсов поможет сократить будущие расходы государства на здравоохранение.</p></abstract><trans-abstract xml:lang="en"><p>Background. Hypertrophic cardiomyopathy (HCM) is one of the most common form of cardiomyopathy in children, with an estimated annual incidence of 2.9 per 100,000 children. The management of patients with HCM requires high healthcare costs. Registry-based medical research is an important tool in assessing health care decision making. Objective. To analyze the costs of the state for the management of pediatric HCM based on the data of the pediatric patient registry. Materials and methods. The study was performed on the basis of the Department of Pediatric Cardiology and Medical Rehabilitation of the Almazov National Medical Research Centre. Currently, the electronic database includes information on 159 children with HCM, including 52 (34 %) girls. The mean age of onset of HCM was 5 years [0; 11], and 68 (44.4 %) children had the onset of myocardial hypertrophy before the age of 1 year. Medical costs included direct medical costs of drug therapy, surgical treatments (implantation of a cardioverter-defibrillator and myectomy) and hospitalization, and indirect costs which included the costs of family caregiving; social benefits due to disability, and death impact on the gross domestic product. Results. The total economic impact of HCM in the context of government spending, is RUB 107.8 million, including medical costs of RUB 27.96 million, direct nonmedical costs of RUB 13.17 million, and indirect costs of RUB 66.7 million. Indirect costs accounted for the lion’s share (61.8 %) of economic impact. Direct medical costs were 26 % of economic impact. The average impact of family caregiving accounted to RUB 11,263.89, and total value were RUB 1.04 million. Conclusion. The intermediate results of our study allow to estimate the economic impact of pediatric HCM to the state. Optimization of financial resource use will help reduce the economic impact on the state to the healthcare sector in the future.  </p></trans-abstract><kwd-group xml:lang="ru"><kwd>внезапная сердечная смерть</kwd><kwd>внутренний валовый продукт</kwd><kwd>гипертрофическая кардиомиопатия</kwd><kwd>регистр пациентов</kwd><kwd>экономические затраты</kwd><kwd>экономический ущерб</kwd></kwd-group><kwd-group xml:lang="en"><kwd>economic costs</kwd><kwd>economic damage</kwd><kwd>gross domestic product</kwd><kwd>hypertrophic cardiomyopathy</kwd><kwd>patient registry</kwd><kwd>sudden cardiac death</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Исследование выполнено при финансовой поддержке Министерства науки и высшего образования Российской Федерации (Соглашение № 075-15-2022-301 от 20.04.2022).</funding-statement><funding-statement xml:lang="en">The research was carried out with the financial support of the Ministry of Science and Higher Education of the Russian Federation (Agreement No. 075-15-2022-301 dated 04/20/2022).</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">Ягудина Р.И., Литвиненко М.М., Сороковиков И.В. 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