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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">lvrach</journal-id><journal-title-group><journal-title xml:lang="ru">Лечащий Врач</journal-title><trans-title-group xml:lang="en"><trans-title>Lechaschi Vrach</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1560-5175</issn><issn pub-type="epub">2687-1181</issn><publisher><publisher-name>ООО «Издательство "Открытые системы"»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.51793/OS.2023.26.12.008</article-id><article-id custom-type="elpub" pub-id-type="custom">lvrach-1166</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>СТРАНИЧКА ПЕДИАТРА</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>PEDIATRICIAN’S PAGE</subject></subj-group></article-categories><title-group><article-title>Проблема дефицита факторов противосвертывающей системы. Клинический пример</article-title><trans-title-group xml:lang="en"><trans-title>The problem of deficiency of factors of the anticoagulant system. Clinical case</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-8311-9506</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гордеева</surname><given-names>О. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Gordeeva</surname><given-names>O. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Гордеева Ольга Борисовна, к.м.н., заведующая отделом научных основ гемостаза; доцент кафедры факультетской педиатрии педиатрического факультета</p><p>119333, Москва, ул. Фотиевой, 10, стр. 1;</p><p>117997, Москва, ул. Островитянова, 1</p><p> </p></bio><bio xml:lang="en"><p>Olga B. Gordeeva, Cand. of Sci. (Med.), Head of the Department of Scientific Fundamentals of Hemostasis; Associate Professor of the Department of Faculty Pediatrics, Faculty of Pediatrics at the ederal</p><p>10 b. 1 Fotieva str., Moscow, 119333;</p><p>1 Ostrovityanova str., Moscow, 117997</p></bio><email xlink:type="simple">obr@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/0000-0001-8116-598X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Доброток</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Dobrotok</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Доброток Альбина Витальевна, сотрудник отдела научных основ гемостаза</p><p>119333, Москва, ул. Фотиевой, 10, стр. 1</p></bio><bio xml:lang="en"><p>Albina V. Dobrotok, employee of the Department of Scientific Fundamentals of Hemostasis</p><p>10 b. 1 Fotieva str., Moscow, 119333</p></bio><email xlink:type="simple">dobrotokav@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/0000-0001-7916-1488</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Андреева</surname><given-names>И. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Andreeva</surname><given-names>I. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Андреева Ирина Вениаминовна, д.м.н., старший научный сотрудник Научно- исследовательского института антимикробной химиотерапии</p><p>214019, Смоленск, ул. Крупской, 28</p></bio><bio xml:lang="en"><p>Irina V. Andreeva, Dr. of Sci. (Med.), Senior Researcher</p><p>28 Krupskaya str., Smolensk, 214019</p></bio><email xlink:type="simple">Irina.Andreeva@antibiotic.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/0000-0002-2655-6192</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Довгань</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Dovgan</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Довгань Евгений Валерьевич, к.м.н., клинический фармаколог</p><p>214018, Смоленск, просп. Гагарина, 27</p></bio><bio xml:lang="en"><p>Evgeniy V. Dovgan, Cand. of Sci. (Med.), clinical pharmacologist</p><p>27 Gagarin ave., Smolensk, 214018</p></bio><email xlink:type="simple">dovganrus@mail.ru</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6316-6046</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Егорова</surname><given-names>О. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Egorova</surname><given-names>O. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Егорова Ольга Анатольвна, к.м.н., доцент кафедры оториноларингологии, заведующая детским оториноларингологическим отделением</p><p>214018, Смоленск, просп. Гагарина, 27</p></bio><bio xml:lang="en"><p>Olga A. Egorova, Cand. of Sci. (Med.), Associate Professor of the Department of Otorhinolaryngology; Head of the Children's Otorhinolaryngology Department</p><p>27 Gagarin ave., Smolensk, 214018</p></bio><email xlink:type="simple">ola-egorova@yandex.ru</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-7584-1467</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Каргин</surname><given-names>В. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Kargin</surname><given-names>V. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Каргин Владимир Сергеевич, к.фарм.н., лаборант-исследователь</p><p>119333, Москва, ул. Фотиевой, 10, стр. 1</p></bio><bio xml:lang="en"><p>Vladimir S. Kargin, Cand. of Sci. (Pharm.), laboratory researcher of the Research Institute of Pediatrics and Children's Health</p><p>10 b. 1 Fotieva str., Moscow, 119333</p></bio><email xlink:type="simple">kvc12.kargin@yandex.ru</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Научно-исследовательский институт педиатрии и охраны здоровья детей Федерального государственного бюджетного научного учреждения Российский научный центр хирургии имени академика Б. В. Петровского; Федеральное государственное автономное образовательное учреждение высшего образования Российский&#13;
национальный исследовательский медицинский университет имени Н. И. Пирогова Министерства&#13;
здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Pediatrics and Children's Health of the Federal State Budgetary Scientific Institution Russian Scientific Center of Surgery named after Academician B. V. Petrovsky; Federal State Autonomous Educational Institution of Higher Education Pirogov Russian National Research Medical University&#13;
of the Ministry of Health of the Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Научно-исследовательский институт педиатрии и охраны здоровья детей Федерального государственного бюджетного научного учреждения Российский научный центр хирургии имени академика Б. В. Петровского</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Pediatrics and Children's Health of the Federal State Budgetary Scientific Institution Russian Scientific Center of Surgery named after Academician B. V. Petrovsky</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Научно-исследовательский институт антимикробной химиотерапии Федерального государственного бюджетного образовательного учреждения высшего образования Смоленский государственный медицинский университет Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Antimicrobial Chemotherapy of the Federal State Budgetary Educational Institution of Higher Education Smolensk State Medical University of the Ministry of Health of the Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>Областное государственное бюджетное учреждение здравоохранения Смоленская областная клиническая&#13;
больница</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Regional State Budgetary Healthcare Institution Smolensk Regional Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2023</year></pub-date><pub-date pub-type="epub"><day>21</day><month>01</month><year>2024</year></pub-date><volume>0</volume><issue>12</issue><fpage>57</fpage><lpage>63</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Гордеева О.Б., Доброток А.В., Андреева И.В., Довгань Е.В., Егорова О.А., Каргин В.С., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Гордеева О.Б., Доброток А.В., Андреева И.В., Довгань Е.В., Егорова О.А., Каргин В.С.</copyright-holder><copyright-holder xml:lang="en">Gordeeva O.B., Dobrotok A.V., Andreeva I.V., Dovgan E.V., Egorova O.A., Kargin V.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://journal.lvrach.ru/jour/article/view/1166">https://journal.lvrach.ru/jour/article/view/1166</self-uri><abstract><sec><title>Введение</title><p>Введение. В доступных зарубежных изданиях имеются сведения о высокой частоте смертности при развитии септических состояний у детей в случае дефицита факторов в системе естественных антикоагулянтов. Известно, что дефицит факторов противосвертывающей системы может быть врожденным и приобретенным. Врожденный дефицит встречается довольно редко, а приобретенный может наблюдаться на фоне воспалительных реакций при хронических, инфекционных заболеваниях, онкопатологии. Особую тревогу представляет отсутствие должного внимания к пациентам детской возрастной категории, клиническая картина тромбоза у которых может быть стертой. При этом именно у детей своевременная диагностика и профилактика, а также выявление риска таких событий могут сыграть ключевую роль в предотвращении инвалидизации. Тяжелыми осложнениями дефицита физиологических антикоагулянтов являются тромботические события и некрозы кожи. Представленный клинический случай описывает эпизод приобретенного дефицита протеина S у ребенка.</p></sec><sec><title>Цель работы</title><p>Цель работы. Провести анализ диагностически сложного клинического случая из реальной врачебной практики с целью привлечения внимания к проблеме дефицита физиологических антикоагулянтов приобретенного характера на фоне бактериальных осложнений после перенесенной вирусной инфекции у детей.</p></sec><sec><title>Методы и методы</title><p>Методы и методы. В статье приводится описание клинического случая приобретенного дефицита фактора противосвертывающей системы, осложненного развитием синдрома диссеминированного внутрисосудистого свертывания, некрозами кожи и тромбозом вен нижних конечностей после перенесенной вирусной инфекции у ребенка младшего возраста. Особенностью данного случая является дебют болезни с поражением кожи конечноестей на фоне развития нарушений в тромбоцитарном и плазменном звене гемостаза при развивающемся дефиците компонентов плазменного звена гемостаза. Для установления диагноза пациенту проведено углубленное исследование системы гемостаза для уточнения дефицита факторов системы естественных антикоагулянтов (протеин С, протеин S, антитромбин III).</p></sec><sec><title>Заключение</title><p>Заключение. Данное клиническое наблюдение показывает значимость тщательного сбора анамнеза и необходимость своевременного обследования пациента при развитии бактериальных осложнений после перенесенной вирусной инфекции. Также клинический случай демонстрирует важность мультидисциплинарного подхода и преемственности между врачами различных специальностей в диагностике тяжелых нарушений свертывающей и противосвертывающих систем, что способствует адекватной оценке выявленных нарушений, прогнозу риска развития возможных осложнений (геморрагического и тромботического синдромов), сохранению качества жизни пациентов.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Background</title><p>Background. In available foreign publications, there is evidence of a high incidence of mortality in the development of septic conditions in children in case of deficiency of factors in the natural anticoagulant system. It is known that deficiency of factors in the anticoagulant system can be congenital and acquired. Congenital deficiency is quite rare, and acquired deficiency can be observed against the background of inflammatory reactions in chronic, infectious diseases, oncopathology. Of particular concern is the lack of proper attention to patients of children's age category, the clinical picture of thrombosis in which may be erased. At the same time, it is in children that timely diagnosis and prevention, as well as identifying the presence of a risk of such events, can play a key role in preventing disability. Severe complications of physiological anticoagulant deficiency are thrombotic events and skin necrosis. The presented clinical case describes an episode of acquired protein S deficiency in a child.</p></sec><sec><title>Objective</title><p>Objective. To analyse a diagnostically complex clinical case from real medical practice in order to draw attention to the problem of acquired physiological anticoagulant deficiency against the background of bacterial complications after viral infection in children.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. The article describes a clinical case of acquired deficiency of the anti-clotting system factor complicated by the development of DIC, skin necrosis and venous thrombosis of the lower limbs after a viral infection in a young child. The peculiarity of this case is the debut of the disease with skin lesions of the extremities against the background of the development of disorders in the platelet and plasma links of haemostasis with a developing deficiency of components of the plasma link of haemostasis. To establish the diagnosis, the patient underwent an in-depth study of the haemostasis system to clarify the deficiency of factors of the natural anticoagulant system (protein C, protein S, antithrombin III).</p></sec><sec><title>Conclusion</title><p>Conclusion. This clinical observation shows the importance of careful history taking and the necessity of timely examination of the patient when bacterial complications develop after a viral infection. The clinical case also demonstrates the importance of multidisciplinary approach and continuity between doctors of different specialities in the diagnosis of severe disorders of coagulation and anti-coagulation systems, which contributes to adequate assessment of the detected disorders, prediction of the risk of possible complications (haemorrhagic and thrombotic syndromes), and preservation of patients' quality of life.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>противосвертывающая система</kwd><kwd>дефициты факторов</kwd><kwd>гемостаз</kwd><kwd>дети</kwd></kwd-group><kwd-group xml:lang="en"><kwd>anti-clotting system</kwd><kwd>factor deficiencies</kwd><kwd>haemostasis</kwd><kwd>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">Sim M. M. S., Wood J. P. 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