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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="en"><front><journal-meta><journal-id journal-id-type="publisher-id">rpcardio</journal-id><journal-title-group><journal-title xml:lang="en">Rational Pharmacotherapy in Cardiology</journal-title><trans-title-group xml:lang="ru"><trans-title>Рациональная Фармакотерапия в Кардиологии</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1819-6446</issn><issn pub-type="epub">2225-3653</issn><publisher><publisher-name>«SILICEA-POLIGRAF» LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.20996/1819-6446-2025-3106</article-id><article-id custom-type="elpub" pub-id-type="custom">rpcardio-3106</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="en"><subject>ORIGINAL STUDIES</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject></subj-group></article-categories><title-group><article-title>Impact of hyperlipoproteinemia(a) on the diagnosis of familial hypercholesterolemia and cardiovascular risk assessment</article-title><trans-title-group xml:lang="ru"><trans-title>Влияние гиперлипопротеидемии(а) на диагноз семейной гиперхолестеринемии и оценку сердечно- сосудистого риска</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5866-506X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Намитоков</surname><given-names>А. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Namitokov</surname><given-names>A. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Алим Муратович Намитоков</p><p>Краснодар</p></bio><bio xml:lang="en"><p>Alim M. Namitokov</p><p>Krasnodar</p></bio><email xlink:type="simple">apakella@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/0000-0003-2760-2792</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чубыкина</surname><given-names>У. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Chubykina</surname><given-names>U. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Ульяна Валерьевна Чубыкина</p><p>Москва</p></bio><bio xml:lang="en"><p>Uliana V. Chubykina</p><p>Moscow</p></bio><email xlink:type="simple">uliankachubykina@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3883-8696</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Зафираки</surname><given-names>В. К.</given-names></name><name name-style="western" xml:lang="en"><surname>Zafiraki</surname><given-names>V. K.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Виталий Константинович Зафираки</p><p>Краснодар</p></bio><bio xml:lang="en"><p>Vitaliy K. Zafiraki</p><p>Krasnodar</p></bio><email xlink:type="simple">vzaphir@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1518-6552</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ежов</surname><given-names>М. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Ezhov</surname><given-names>M. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Марат Владиславович Ежов</p><p>Москва</p></bio><bio xml:lang="en"><p>Marat V. Ezhov</p><p>Moscow</p></bio><email xlink:type="simple">marat_ezhov@mail.ru</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ГБУЗ "Научно- исследовательский институт — Краевая клиническая больница № 1 им. С. В. Очаповского" Минздрава Краснодарского края; ФГБОУ ВО "Кубанский государственный медицинский университет" Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Scientific Research Institute — S. V. Ochapovsky Regional Clinical Hospital No 1; Kuban State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФГБУ "Национальный медицинский исследовательский центр кардиологии им. акад. Е. И. Чазова" Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>E. I. Chazov National Medical Research Center of Cardiology</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>ФГБОУ ВО "Кубанский государственный медицинский университет" Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Kuban State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2025</year></pub-date><pub-date pub-type="epub"><day>19</day><month>03</month><year>2025</year></pub-date><volume>21</volume><issue>1</issue><fpage>47</fpage><lpage>53</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Namitokov A.M., Chubykina U.V., Zafiraki V.K., Ezhov M.V., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Намитоков А.М., Чубыкина У.В., Зафираки В.К., Ежов М.В.</copyright-holder><copyright-holder xml:lang="en">Namitokov A.M., Chubykina U.V., Zafiraki V.K., Ezhov M.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.rpcardio.online/jour/article/view/3106">https://www.rpcardio.online/jour/article/view/3106</self-uri><abstract><sec><title>   Aim</title><p>   Aim. To assess the contribution of extremely elevated Lipoprotein(a) [Lp(a)] level to cardiovascular risk stratification using the SCORE-2 scale and the likelihood of familial hypercholesterolemia (FH) diagnosis.</p></sec><sec><title>   Material and methods</title><p>   Material and methods. A retrospective cohort study included 45 patients (25 men) with hyperlipoproteinemia(a) from the federal registry "RENESSANS." Participants were over 40 years old and had Lp(a) levels ≥180 mg/dL. The diagnosis of heterozygous FH was confirmed using the Dutch Lipid Clinic Network (DLCN) criteria. Low-density lipoprotein cholesterol (LDL-C) levels were adjusted using Friedewald formula modified by Dahlen. Cardiovascular risk (SCORE-2) was calculated considering LDL-C levels before and after adjustment.</p></sec><sec><title>   Results</title><p>   Results. Among the 45 patients, the LDL-C level calculated using Friedewald formula was 5.1 [3.17; 7.19] mmol/L. After adjustment with Dahlen’s modification, the LDL-C level decreased to 3.03 [1.54; 4.96] mmol/L, representing a significant reduction (by 2.07 mmol/L or 40.6 %, p &lt; 0.001). Adjusted LDL-C levels affected the FH diagnosis in 8 (26 %) out of 31 patients. Incorporating Lp(a) into the SCORE-2 risk assessment significantly increased cardiovascular risk estimates. The median SCORE-2 value initially was 14 % [7; 20], which increased to 27.2 % [13.6; 54.5] after adjustment, representing a 94 % increase (p &lt; 0.005).</p></sec><sec><title>   Conclusion</title><p>   Conclusion. Including Lp(a) in routine cardiovascular risk assessment and FH diagnosis is an important addition for more accurate risk stratification and appropriate pharmacological management. In patients with extreme hyperlipoproteinemia(a), LDL-C levels may be overestimated compared to true values, potentially affecting the accuracy of FH diagnosis and treatment strategies. Future multicenter studies should include larger and more diverse populations to validate these findings.</p></sec></abstract><trans-abstract xml:lang="ru"><sec><title>   Цель</title><p>   Цель. Оценить вклад экстремально повышенной концентрации липопротеида(а) (Лп(а)) в стратификацию сердечно-сосудистого риска по шкале SCORE-2 и вероятность диагноза семейной гиперхолестеринемии (СГХС).</p></sec><sec><title>   Материал и методы</title><p>   Материал и методы. В ретроспективное исследование включены 45 пациентов (из них 25 мужчин) с гиперлипопротеидемией (а) из федерального регистра "РЕНЕССАНС". Участники исследования были старше 40 лет и имели уровень Лп(а) ≥180 мг/дл. Диагноз гетерозиготной СГХС подтвержден с использованием критериев сети голландских липидных клиник (Dutch Lipid Clinic Network, DLCN). Корректировка уровня холестерина липопротеидов низкой плотности (ХС ЛНП) выполнена по формуле Фридвальда в модификации Далена. Сердечно- сосудистый риск (по шкале SCORE2) рассчитывался с учётом уровня ХС ЛНП до и после проведённой коррекции.</p></sec><sec><title>   Результаты</title><p>   Результаты. Среди 45 пациентов уровень ХС ЛНП, рассчитанный по формуле Фридвальда, составил 5,1 [3,17; 7,19] ммоль/л. После корректировки с использованием модификации Далена уровень ХС ЛНП составил 3,03 [1,54; 4,96] ммоль/л, т. е. оказался значительно ниже исходного (на 2,07 ммоль/л и на 40,6 %, p &lt; 0,001). Пересчёт на основе скорректированных уровней ХС ЛНП повлиял на диагноз СГХС у 8 (26 %) из 31 пациентов. Корректировка значений риска по SCORE2 с учетом Лп(а) показала значительное увеличение величины сердечно- сосудистого риска. Исходная медиана значения SCORE2 составила 14 % [7; 20], а после корректировки увеличилась до 27,2 % [13,6; 54,5] — на 94 % (p &lt; 0,005).</p></sec><sec><title>   Заключение</title><p>   Заключение. Включение Лп(а) в рутинную оценку сердечно- сосудистого риска и диагностику СГХС является важным дополнением для более точной стратификации риска и назначения медикаментозного лечения. У пациентов с экстремальной гиперлипопротеидемией (а) уровень ХС ЛНП может быть завышенным в сравнении с истинным, что неизбежно повлияет на точность диагноза СГХС и стратегию лечения. Будущие многоцентровые исследования должны включать более крупные и разнообразные группы населения для подтверждения этих выводов.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>липопротеид(а)</kwd><kwd>семейная гиперхолестеринемия</kwd><kwd>SCORE-2</kwd><kwd>формула Фридвальда</kwd><kwd>поправка Далена</kwd><kwd>гиперлипопротеидемия(а)</kwd><kwd>липидный профиль</kwd></kwd-group><kwd-group xml:lang="en"><kwd>lipoprotein (a)</kwd><kwd>familial hypercholesterolemia</kwd><kwd>SCORE-2</kwd><kwd>Friedwald formula</kwd><kwd>Dahlen’s correction</kwd><kwd>hyperlipoproteinemia (a)</kwd><kwd>lipid profile</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Нет</funding-statement><funding-statement xml:lang="en">None</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">Berg K. A new serum type system in man — the Lp system. 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