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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">clinmed</journal-id><journal-title-group><journal-title xml:lang="ru">Клиническая медицина</journal-title><trans-title-group xml:lang="en"><trans-title>Clinical Medicine (Russian Journal)</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">0023-2149</issn><issn pub-type="epub">2412-1339</issn><publisher><publisher-name>ООО «Медицинское информационное агентство»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.30629/0023-2149-2024-102-8-655-660</article-id><article-id custom-type="elpub" pub-id-type="custom">clinmed-927</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>ЗАМЕТКИ И НАБЛЮДЕНИЯ ИЗ ПРАКТИКИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>NOTES AND OBSERVATIONS FROM PRACTICE</subject></subj-group></article-categories><title-group><article-title>Новая тромбофилия в результате мутации фибриногена</article-title><trans-title-group xml:lang="en"><trans-title>New thrombophylia as a result of fibrinogen mutation</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1453-7290</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хлевчук</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Khlevchuk</surname><given-names>T. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Хлевчук Татьяна Васильевна — канд. мед. наук, доцент кафедры госпитальной терапии №1 ИКМ им. Н.В. Склифосовского</p><p>Москва </p></bio><bio xml:lang="en"><p>Tatyana V. Khlevchuk — Candidate of Medical Sciences, Associate Professor of the Department of Hospital Therapy No. 1</p><p>Moscow</p></bio><email xlink:type="simple">khlevchuk@inbox.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/0000-0002-3135-3680</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Старжевская</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Starzhevskaya</surname><given-names>A. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Старжевская Анастасия Александровна — аспирант кафедры госпитальной терапии №1</p><p>Москва</p></bio><bio xml:lang="en"><p>Anastasia A. Starzhevskaya — postgraduate student of the Department of Hospital Therapy No. 1</p><p>Moscow</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0002-8499-1077</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Рогова</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Rogova</surname><given-names>E. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Рогова Елизавета Андреевна — студентка 6-го курса </p><p>Москва</p></bio><bio xml:lang="en"><p>Elizaveta A. Rogova — a 6th—year student </p><p>Moscow</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2161-2140</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Агеев</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Ageev</surname><given-names>A. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Агеев Антон Александрович — ассистент кафедры госпитальной терапии №1 </p><p>Москва</p></bio><bio xml:lang="en"><p>Anton A. Ageev — Assistant of the Department of Hospital Therapy No. 1 </p><p>Moscow</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7938-1763</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кондратьева</surname><given-names>Т. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Kondratyeva</surname><given-names>T. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кондратьева Татьяна Борисовна — канд. мед. наук, доцент кафедры госпитальной терапии №1 </p><p>Москва</p></bio><bio xml:lang="en"><p>Tatyana B. Kondratyeva — Candidate of Medical Sciences, Associate professor of the Department of Hospital Therapy No. 1</p><p>Moscow</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4802-6544</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Аксенова</surname><given-names>М. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Aksenova</surname><given-names>M. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Аксенова Марианна Борисовна — канд. мед. наук, доцент кафедры госпитальной терапии №1</p><p>Москва</p></bio><bio xml:lang="en"><p>Marianna B. Aksenova — Candidate of Medical Sciences, Associate Professor of the Department of Hospital Therapy No. 1</p><p>Moscow</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3979-5122</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Попов</surname><given-names>Р. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Popov</surname><given-names>R. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Попов Ростислав Григорьевич — студент 3-го курса</p><p>Москва</p></bio><bio xml:lang="en"><p>Rostislav G. Popov – a 3rd year student</p><p>Moscow</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0004-3822-818X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Карпова</surname><given-names>П. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Karpova</surname><given-names>Р. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Карпова Полина Андреевна — студентка 3-го курса</p><p>Москва </p></bio><bio xml:lang="en"><p>Polina A. Karpova — 3rd year student </p><p>Moscow</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3496-6466</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Попова</surname><given-names>Л. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Popova</surname><given-names>L. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Попова Людмила Викторовна — канд. мед. наук, доцент кафедры госпитальной терапии №1</p><p>Москва</p></bio><bio xml:lang="en"><p>Lyudmila V. Popova — Candidate of Medical Sciences, Associate Professor of the Department of Hospital Therapy No. 1 </p><p>Moscow</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Институт клинической медицины им. Н.В. Склифосовского; Всеросийская Ассоциация по изучению тромбозов, гемостаза и патологии сосудов им. А.А. Шмидта–Б.А. Кудряшова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of Russia (Sechenov University), Institute of Clinical Medicine named after N.V. Sklifosovsky; Russian Association on Thrombosis, Hemostasis and Vascular Pathology A.A. Schmidt–B.A. Kudryashov</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Институт клинической медицины им. Н.В. Склифосовского</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of Russia (Sechenov University), Institute of Clinical Medicine named after N.V. Sklifosovsky</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>12</day><month>01</month><year>2025</year></pub-date><volume>102</volume><issue>8</issue><fpage>655</fpage><lpage>660</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Хлевчук Т.В., Старжевская А.А., Рогова Е.А., Агеев А.А., Кондратьева Т.Б., Аксенова М.Б., Попов Р.Г., Карпова П.А., Попова Л.В., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Хлевчук Т.В., Старжевская А.А., Рогова Е.А., Агеев А.А., Кондратьева Т.Б., Аксенова М.Б., Попов Р.Г., Карпова П.А., Попова Л.В.</copyright-holder><copyright-holder xml:lang="en">Khlevchuk T.V., Starzhevskaya A.A., Rogova E.A., Ageev A.A., Kondratyeva T.V., Aksenova M.B., Popov R.G., Karpova Р.A., Popova L.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.clinmedjournal.com/jour/article/view/927">https://www.clinmedjournal.com/jour/article/view/927</self-uri><abstract><p>Фибриноген (ФГ) — фактор I свертывающей системы крови — важное звено каскада гемокоагуляции. Его основная физиологическая функция — участие в заключительной стадии процесса свертывания крови, когда растворимый фибриноген под действием тромбина и фактора XII превращается в нерастворимый фибрин. Появляется все больше данных о врожденных нарушениях синтеза и/или структуры фибриногена, дефицит которого представлен в 9,3 % случаев редких нарушений свертываемости крови. Разнообразие фенотипов при врожденном нарушении структуры ФГ, проявляющееся склонностью к кровоточивости и/или тромбозам, преимущественно в артериальном русле (инфаркты, инсульты), определяет современные трудности диагностики. В связи с этим необходимо подробнее исследовать дефекты ФГ у пациентов. В нашей работе представлен обзор современной литературы о вкладе фибриногена в патогенез сердечно-сосудистых заболеваний. В качестве подтверждения данной информации приведен клинический случай молодой женщины 36 лет, у которой развитие инфаркта миокарда без обструктивного поражения коронарных артерий произошло на фоне носительства гомозиготной мутации фибриногена (FGB −455 G/A).</p></abstract><trans-abstract xml:lang="en"><p>Fibrinogen (FG), factor I of the blood coagulation system, is a critical component of the hemostatic cascade. Its primary physiological function involves the final stage of blood coagulation, where soluble fibrinogen is converted into insoluble fibrin by thrombin and factor XII. Increasing data highlight congenital disorders of fibrinogen synthesis and/or structure, with deficiencies found in 9.3% of rare blood coagulation disorders. The variety of phenotypes in congenital FG structural disorders, characterized by a tendency for bleeding and/or thrombosis, primarily in the arterial system (infarcts, strokes), complicates modern diagnosis. Therefore, a detailed study of FG defects in patients is required. This article reviews current literature on the role of fibrinogen in the pathogenesis of cardiovascular diseases. A clinical case is presented involving a 36-year-old woman who experienced a myocardial infarction without obstructive coronary artery disease, linked to a homozygous mutation in fibrinogen (FGB −455 G/A).</p></trans-abstract><kwd-group xml:lang="ru"><kwd>инфаркт миокарда без обструктивного поражения коронарных артерий</kwd><kwd>оральные контрацептивы</kwd><kwd>тромбоз</kwd><kwd>мутация фибриногена FGB −455 G/A</kwd></kwd-group><kwd-group xml:lang="en"><kwd>myocardial infarction without obstructive coronary artery disease</kwd><kwd>oral contraceptives</kwd><kwd>thrombosis</kwd><kwd>fibrinogen  mutation FGB −455 G/A</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">Vilar R., Fish R.J., Casini A., Neerman-Arbez M. Fibrin(ogen) in human disease: both friend and foe. Haematologica. 2020;105(2):284–296. 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