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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">ateroskleroz</journal-id><journal-title-group><journal-title xml:lang="ru">Атеросклероз</journal-title><trans-title-group xml:lang="en"><trans-title>Ateroscleroz</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2078-256X</issn><issn pub-type="epub">2949-3633</issn><publisher><publisher-name>НИИТПМ-филиал ИЦиГ СО РАН</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.52727/2078-256X-2022-18-4-302-316</article-id><article-id custom-type="elpub" pub-id-type="custom">ateroskleroz-858</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>Распространенность и клиническая характеристика фенотипов семейной гиперхолестеринемии у пациентов с острым коронарным синдромом: данные проспективного наблюдательного регистрового исследования пациентов с острым коронарным синдромом (ПРИМА-ОКС)</article-title><trans-title-group xml:lang="en"><trans-title>Prevalence and clinical characteristics of familial hypercholesterolemia phenotypes in patients with acute coronary syndrome: data from the prospective observational registry study of patients with acute coronary syndrome (PRIMA-ACS)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-0108-5996</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Усова</surname><given-names>Е. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Usova</surname><given-names>E. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Елена Ивановна Усова, врач-кардиолог, аспирант кафедры кардиологии факультета подготовки кадров высшей квалификации Института медицинского образования, младший научный сотрудник НИЛ нарушений липидного обмена и атеросклероза НЦМУ «Центр персонализированной медицины»</p><p>197341, г. Санкт-Перербург, ул. Аккуратова, 2</p></bio><bio xml:lang="en"><p>Elena I. Usova, junior reseahcher, postgraduate student of the Department of Cardiology, Faculty of Advanced Training of the Institute of Medical Education, junior researcher, research laboratory of lipid disorders and atherosclerosis WCRC for Personalized Medicine</p><p>2, Akkuratov str., Saint Petersburg, 197341</p></bio><email xlink:type="simple">el.lenkin@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/0000-0002-1025-3728</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Малишевский</surname><given-names>Л. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Malishevskii</surname><given-names>L. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Лев Михайлович Малишевский, аспирант кафедры сердечно-сосудистой хирургии факультета подготовки кадров высшей квалификации Института медицинского образования</p><p>197341, г. Санкт-Перербург, ул. Аккуратова, 2</p></bio><bio xml:lang="en"><p>Lev M. Malishevskii, postgraduate student of the Department of Cardiovascular Surgery, Faculty of Advanced Training of the Institute of Medical Education</p><p>2, Akkuratov str., Saint Petersburg, 197341</p></bio><email xlink:type="simple">levmalishevskii@gmail.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/0000-0003-3878-961X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Алиева</surname><given-names>М. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Alieva</surname><given-names>M. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Мадина Сайгидовна Алиева, врач-анестезиолог-реаниматолог</p><p>197341, г. Санкт-Перербург, ул. Аккуратова, 2</p></bio><bio xml:lang="en"><p>Madina S. Alieva, anesthesiologist-resuscitator</p><p>2, Akkuratov str., Saint Petersburg, 197341</p></bio><email xlink:type="simple">alieva_ms@almazovcentre.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/0000-0001-5656-3978</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Яковлев</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Yakovlev</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Алексей Николаевич Яковлев, канд. мед. наук, начальник службы по развитию регионального здравоохранения Управления по реализации федеральных проектов, зав. НИЛ технологий прогнозирования риска развития сердечно-сосудистых осложнений НЦМУ «Центр персонализированной медицины»</p><p>197341, г. Санкт-Перербург, ул. Аккуратова, 2</p></bio><bio xml:lang="en"><p>Alexey N. Yakovlev, candidate of medical sciences, head of the Healthcare Development Service at the division of the Federal projects implementation, head of the Research Laboratory for Cardiovascular Risk Prediction Technologies WCRC for Personalized Medicine</p><p>2, Akkuratov str., Saint Petersburg, 197341</p></bio><email xlink:type="simple">alex.yakovlev@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/0000-0002-9845-331X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Алиева</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Alieva</surname><given-names>A. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Асият Сайгидовна Алиева, канд. мед. наук, врач-кардиолог, зав. НИЛ нарушений липидного обмена и атеросклероза НЦМУ «Центр персонализированной медицины», руководитель Центра Атеросклероза и нарушений липидного обмена</p><p>197341, г. Санкт-Перербург, ул. Аккуратова, 2</p></bio><bio xml:lang="en"><p>Asiiat S. Alieva, candidate of medical sciences, head of laboratory of lipid disorders and atherosclerosis WCRC for Personalized Medicine, head of the Center for Atherosclerosis and Lipid Disorders</p><p>2, Akkuratov str., Saint Petersburg, 197341</p></bio><email xlink:type="simple">asiiat.alieva.s@gmail.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/0000-0001-8169-7812</contrib-id><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>Александра Олеговна Конради, д-р мед наук, проф., академик РАН, зав. НИО артериальной гипертензии, зав. кафедрой организации управления и экономики здравоохранения Института медицинского образования, зам. генерального директора по научной работе</p><p>197341, г. Санкт-Перербург, ул. Аккуратова, 2</p></bio><bio xml:lang="en"><p>Alexandra O. Konradi, doctor of medical sciences, professor, RAS academician, Head of the Research Institute of Arterial Hypertension, Head of the Department of Organization of Management and Economics of Health Care, Institute of Medical Education, Deputy General director</p><p>2, Akkuratov str., Saint Petersburg, 197341</p></bio><email xlink:type="simple">ahleague@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru">Федеральное государственное бюджетное учреждение &#13;
«Национальный медицинский исследовательский центр им. В.А. Алмазова» Министерства здравоохранения Российской Федерации<country>Россия</country></aff><aff xml:lang="en">Almazov National Medical Research Centre<country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2022</year></pub-date><pub-date pub-type="epub"><day>18</day><month>01</month><year>2023</year></pub-date><volume>18</volume><issue>4</issue><fpage>302</fpage><lpage>316</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Усова Е.И., Малишевский Л.В., Алиева М.С., Яковлев А.Н., Алиева А.С., Конради А.О., 2023</copyright-statement><copyright-year>2023</copyright-year><copyright-holder xml:lang="ru">Усова Е.И., Малишевский Л.В., Алиева М.С., Яковлев А.Н., Алиева А.С., Конради А.О.</copyright-holder><copyright-holder xml:lang="en">Usova E.I., Malishevskii L.M., Alieva M.S., Yakovlev A.N., Alieva A.S., Konradi A.O.</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://ateroskleroz.elpub.ru/jour/article/view/858">https://ateroskleroz.elpub.ru/jour/article/view/858</self-uri><abstract><p>Данные о распространенности и клинических особенностях семейной гиперхолестеринемии (СГХС) в когорте пациентов с острым коронарным синдромом (ОКС) немногочисленны и представляют собой особый интерес в связи с высоким риском развития повторных сердечно-сосудистых событий. Цель исследования заключалась в анализе распространенности и оценке клинических параметров лиц с фенотипической СГХС среди пациентов с ОКС с оценкой развития повторных сердечно-сосудистых событий.</p><sec><title>Материал и методы</title><p>Материал и методы. В исследование включено 498 пациентов, госпитализированных в связи с развитием ОКС. Клиническую вероятность наличия СГХС оценивали с помощью диагностических критериев Голландских липидных клиник (DLCN score). Наблюдение длилось в течение одного года.</p></sec><sec><title>Результаты</title><p>Результаты. У 86 (17,3 %) пациентов определен возможный диагноз СГХС, у 6 (1,2 %) – вероятный диагноз СГХС. По сравнению с лицами без клинической СГХС больные с возможной и вероятной СГХС характеризовались более молодым возрастом (соответственно 68,5, 59,5 и 50,5 года, p &lt; 0,001), высокой частотой встречаемости семейного анамнеза преждевременных сердечно-сосудистых заболеваний (ССЗ) (соответственно 22,9, 77,9 и 83,3 %, p &lt; 0,001) и ранним персональным анамнезом ССЗ (соответственно 24,1, 90,7 и 100 %, p &lt; 0,001). Пациенты с СГХС имели более значимые нарушения липидного обмена: сывороточный уровень общего холестерина и холестерина липопротеинов низкой плотности был значимо выше у лиц с возможной и вероятной СГХС (p &lt; 0,001) по сравнению с пациентами без СГХС. Статистически значимых различий в развитии сердечно-сосудистых событий в период госпитализации и в первый год после выписки из стационара не наблюдалось (p &gt; 0,05).</p></sec><sec><title>Заключение</title><p>Заключение. Выявлена высокая распространенность клинической СГХС в популяции пациентов с ОКС. Лица с СГХС характеризуются более молодым возрастом, высокой частотой встречаемости семейного анамнеза преждевременных ССЗ, ранним персональным анамнезом ССЗ и более значимыми нарушениями липидного обмена. Полученные данные демонстрируют важность выявления пациентов с СГХС на догоспитальном этапе с целью интенсификации гиполипидемической терапии для снижения атерогенной нагрузки, предотвращения развитие острых первичных и повторных сердечно-сосудистых событий.</p></sec></abstract><trans-abstract xml:lang="en"><p>Data on the prevalence and clinical features of familial hypercholesterolemia (FH) in the cohort of patients with acute coronary syndrome (ACS) are few and of particular interest due to the high risk of recurrent cardiovascular events. The aim of the study was to analyze the prevalence and evaluate the clinical parameters of patients with phenotypic FH among ACS patients with an assessment of the development of recurrent cardiovascular events.</p><sec><title>Material and methods</title><p>Material and methods. The study included 498 patients hospitalized for the development of ACS. The clinical probability of FH was assessed using the diagnostic criteria of the Dutch Lipid Clinics (DLCN score). The follow-up lasted for one year.</p></sec><sec><title>Results</title><p>Results. A possible diagnosis of FH was determined in 86 (17.3 %) patients, and a probable diagnosis of FH was determined in 6 (1.2 %) patients. Compared with the group of patients without clinical FH, patients with possible and probable FH were characterized by younger age (68.5 years versus 59.5 and 50.5 years, p &lt; 0.001), higher incidence of family history of premature cardiovascular diseases (CVDs) (22.9 % versus 77.9 % and 83.3 %, p &lt; 0.001) and earlier personal history of CVDs (24.1 % versus 90.7 and 100 %, p &lt; 0.001). Patients with FH had more significant abnormalities of lipid metabolism: level of total serum cholesterol and low-density lipoprotein cholesterol was significantly higher among patients with possible and probable FH (p &lt; 0.001) compared to patients without FH. There were no statistically significant differences in the development of cardiovascular events during hospitalization and in the first year after hospital discharge (p &gt; 0.05).</p></sec><sec><title>Conclusions</title><p>Conclusions. A high prevalence of clinical FH in the population of ACS patients has been revealed. Patients with FH are characterized by younger age, high frequency of family history of premature CVD, early personal history of CVD and more significant lipid disorders. The obtained data demonstrate the importance of identifying patients with FH in order to intensify lipid-lowering therapy to reduce atherogenic burden and prevent the development of acute primary and recurrent cardiovascular events.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>семейная гиперхолестеринемия</kwd><kwd>острый коронарный синдром</kwd><kwd>распространенность</kwd><kwd>повторные сердечно-сосудистые события</kwd></kwd-group><kwd-group xml:lang="en"><kwd>familial hypercholesterolemia</kwd><kwd>acute coronary syndrome</kwd><kwd>prevalence</kwd><kwd>recurrent cardiovascular events</kwd></kwd-group><funding-group xml:lang="ru"><funding-statement>Исследование выполнено при финансовой поддержке Министерства науки и высшего образования Российской Федерации (Соглашение № 075-15-2022-301 от  20.04.2022).</funding-statement></funding-group><funding-group xml:lang="en"><funding-statement>This work was financially supported by the Ministry of Science and Higher Education of the Russian Federation (Agreement No. 075-15-2022-301).</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">Foody J.M. 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