<?xml version="1.0" encoding="UTF-8"?>
<!DOCTYPE article PUBLIC "-//NLM//DTD JATS (Z39.96) Journal Publishing DTD v1.3 20210610//EN" "JATS-journalpublishing1-3.dtd">
<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">bloodjour</journal-id><journal-title-group><journal-title xml:lang="ru">Гематология и трансфузиология</journal-title><trans-title-group xml:lang="en"><trans-title>Russian journal of hematology and transfusiology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">0234-5730</issn><issn pub-type="epub">2411-3042</issn><publisher><publisher-name>ООО Издательский дом «Практика»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.35754/0234-5730-2019-64-3-297-316</article-id><article-id custom-type="elpub" pub-id-type="custom">bloodjour-149</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>ПРИМЕНЕНИЕ РОТАЦИОННОЙ ТРОМБОЭЛАСТОМЕТРИИ ДЛЯ ДИАГНОСТИКИ ДЕФИЦИТА ФАКТОРОВ СВЕРТЫВАНИЯ И КОНТРОЛЯ ГЕМОСТАТИЧЕСКОЙ ТЕРАПИИ У БОЛЬНЫХ НАСЛЕДСТВЕННЫМИ КОАГУЛОПАТИЯМИ</article-title><trans-title-group xml:lang="en"><trans-title>ROTATION THROMBOELASTOMETRY FOR THE DIAGNOSIS OF FACTOR DEFICIENCY AND MANAGEMENT OF THE HEMOSTATIC THERAPY IN PATIENTS WITH INHERITED COAGULATION DISORDERS</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-8818-8949</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Галстян</surname><given-names>Г. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Galstyan</surname><given-names>G. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>доктор медицинских наук, заведующий отделением реанимации и интенсивной терапии,</p><p>125167, Москва</p></bio><bio xml:lang="en"><p>Dr. Sci. (Med.), Head of the Resuscitation and Intensive Care Unit,</p><p>125167, Moscow</p></bio><email xlink:type="simple">gengalst@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/0000-0002-7783-5861</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Полеводова</surname><given-names>О. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Polevodova</surname><given-names>O. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-анестезиолог-реаниматолог отделения реанимации и интенсивной терапии,</p><p>125167, Москва</p></bio><bio xml:lang="en"><p>Intensivist, Resuscitation and Intensive Care Unit,</p><p>125167, Moscow</p></bio><email xlink:type="simple">polevodova_md@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/0000-0002-6991-7437</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Яковлева</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Yakovleva</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>кандидат медицинских наук, врач-гематолог отдела коагулопатий,</p><p>125167, Москва</p></bio><bio xml:lang="en"><p>Cand. Sci. (Med.), Hematologist, Coagulopathy Department,</p><p>125167, Moscow</p></bio><email xlink:type="simple">hemophilia2012@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/0000-0002-7916-2322</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Щекина</surname><given-names>А. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Shchekina</surname><given-names>A. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>аспирант отделения реанимации и интенсивной терапии,</p><p>125167, Москва</p></bio><bio xml:lang="en"><p>Postgraduate Researcher, Resuscitation and Intensive Care Unit,</p><p>125167, Moscow</p></bio><email xlink:type="simple">shekina_ae@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>National Research Center for Hematology</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2019</year></pub-date><pub-date pub-type="epub"><day>09</day><month>11</month><year>2019</year></pub-date><volume>64</volume><issue>3</issue><fpage>297</fpage><lpage>316</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Галстян Г.М., Полеводова О.А., Яковлева Е.В., Щекина А.Е., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Галстян Г.М., Полеводова О.А., Яковлева Е.В., Щекина А.Е.</copyright-holder><copyright-holder xml:lang="en">Galstyan G.M., Polevodova O.A., Yakovleva E.V., Shchekina A.E.</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.htjournal.ru/jour/article/view/149">https://www.htjournal.ru/jour/article/view/149</self-uri><abstract><sec><title>Введение</title><p>Введение. Ротационная тромбоэластометрия (РОТЭМ) является методом исследования гемостаза, который выполняется по месту лечения и позволяет выявлять нарушения по внешнему и/или внутреннему пути свертывания крови.</p><p>Цель работы — изучить возможность использования РОТЭМ для диагностики дефицита отдельных факторов свертывания крови и мониторинга эффективности и безопасности гемостатической терапии, проводимой при этих коагулопатиях.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. В исследование было включено 9 больных с врожденным дефицитом различных факторов свертывания крови. Проводили коагулогические тесты (определение активированного частичного тромбопластинового времени, протромбина по Квику, фибриногена, плазменной активности факторов свертывания FV, FVII, FVIII, FIX, FXI и FXII) и РОТЭМ. Для исключения влияния гепарина или гипофибриногенемии выполняли HEPTEM и/или FIBTEM. Для выявления дефицита отдельных факторов свертывания крови использовали образцы стандартной плазмы и плазмы, дефицитной по одному из факторов свертывания крови. Исследуемую цитратную кровь смешивали в соотношении 2:1 со стандартной плазмой и с дефицитной по одному из исследуемых факторов плазмой, повторно выполняли тесты EXTEM и/или INTEM. Дефицит фактора свертывания крови подтверждали по изменению параметра CT.</p></sec><sec><title>Результаты</title><p>Результаты. Изолированное удлинение CT в тесте EXTEM выявлялось при дефиците FVII, удлинение CT только в INTEM возникало при дефиците FVIII, FIX, FXI, FXII, одновременное удлинение EXTEM и INTEM определялось при дефиците FV. При выполнении пробы со смесью цитратной крови и стандартной плазмы параметр CT укорачивался в тесте EXTEM при дефиците FVII, в тесте INTEM — при дефиците FVIII, FIX, FXI, FXII, в обоих тестах — при дефиците FV. В пробе со смесью цельной крови и дефицитной по исследуемому фактору плазмой интервал CT сохранялся удлиненным либо увеличивался. Приведены клинические примеры диагностики дефицита факторов свертывания с помощью РОТЭМ.</p></sec><sec><title>Заключение</title><p>Заключение. Нормализация показателей РОТЭМ в пробе со стандартной плазмой и сохранение гипокоагуляции в пробе с дефицитной по фактору плазмой позволяет подтвердить дефицит фактора свертывания крови. С помощью РОТЭМ возможно проведение мониторинга эффективности гемостатической терапии при врожденных дефицитах отдельных факторов свертывания крови. </p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. Rotation thromboelastometry (ROTEM) is a method for studying haemostasis at the place of providing care, which allows identification of disorders on the basis of extrinsic and / or intrinsic coagulation pathways.</p></sec><sec><title>Aim</title><p>Aim. To study the possibility of using ROTEM for diagnosing the deficiency of individual blood coagulation factors, as well as for monitoring the efficacy and safety of haemostatic therapy in such coagulopathies.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. The study included 9 patients with a congenital deficiency in various coagulation factors. The authors performed ROTEM, as well as coagulological tests to determine activated partial thromboplastin time, Quick’s value, fibrinogen and the plasma activity of coagulation factors FV, FVII, FVIII, FIX, FXI and FXII. In order to exclude the effects of heparin or hyperfibrinogenaemia, HEPTEM and / or FIBTEM were conducted. In order to identify the deficiency of individual coagulation factors, the authors used standard plasma samples and plasma deficient in one of the coagulation factors. The citrated blood under study was mixed with standard plasma and that deficient in one of the studied factors in a 2:1 ratio for subsequent repetition of the EXTEM and / or INTEM tests. Coagulation factor deficiency was confirmed by a change in the CT parameter.</p></sec><sec><title>Results</title><p>Results. Isolated prolongation of EXTEM CT was detected in the setting of FVII deficiency; while INTEM CT prolongation occurred under the deficiency of FVIII, FIX, FXI, FXII, with simultaneous EXTEM and INTEM CT prolongation being observed in the context of FVII deficiency. The test of a mixture of citrated blood and standard plasma revealed shortened EXTEM CT in the setting of FVII deficiency and shortened INTEM CT under the deficiency of FVIII, FIX, FXI, FXII, with the shortening of both EXTEM and INTEM CT being detected in the setting of FV deficiency. In a sample containing a mixture of whole blood and plasma deficient in the studied factor, CT remained prolonged or increased. Clinical examples of using ROTEM for diagnosing coagulation factor deficiency are presented.</p></sec><sec><title>Conclusion</title><p>Conclusion. The normalisation of ROTEM parameters in a standard plasma sample along with the preservation of hypocoagulation in a factor-deficient plasma sample confirms the coagulation factor deficiency. ROTEM provides the possibility of monitoring the efficacy of haemostatic therapy for congenital deficiencies in individual blood coagulation factors. </p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>ротационная тромбоэластометрия</kwd><kwd>дефицит факторов свертывания</kwd><kwd>наследственная коагулопатия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>rotation thromboelastometry</kwd><kwd>inherited coagulopathy</kwd><kwd>coagulation factor deficiency</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">Saner F.H., Kirchner C. Monitoring and Treatment of Coagulation Disorders in End-Stage Liver Disease. Visc Med. 2016; 32(4): 241–8.</mixed-citation><mixed-citation xml:lang="en">Saner F.H., Kirchner C. Monitoring and Treatment of Coagulation Disorders in End-Stage Liver Disease. Visc Med. 2016; 32(4): 241–8.</mixed-citation></citation-alternatives></ref><ref id="cit2"><label>2</label><citation-alternatives><mixed-citation xml:lang="ru">Дементьева И.И., Морозов Ю.А., Чарная М.А., Гончарова А.В. Технологии point of care в клинике неотложных состояний. Клиническая лабораторная диагностика. 2013; (7): 5–10.</mixed-citation><mixed-citation xml:lang="en">Dementiyeva I.I., Morozov Yu.A., Tcharnaya M.A., Gontcharova A.V. The point-of-care technologies in clinic of emergency states. Klinicheskaya laboratornaya diagnostika. 2013; (7): 5–10 (In Russian).</mixed-citation></citation-alternatives></ref><ref id="cit3"><label>3</label><citation-alternatives><mixed-citation xml:lang="ru">Lang T., Bauters A., Braun S.L. et al. Multi-centre investigation on reference ranges for ROTEM thromboelastometry. Blood coagulation &amp; fi brinolysis: Int. J Haemost Thromb. 2005; 16(4): 301–10.</mixed-citation><mixed-citation xml:lang="en">Lang T., Bauters A., Braun S.L. et al. Multi-centre investigation on reference ranges for ROTEM thromboelastometry. Blood coagulation &amp; fi brinolysis: Int. J Haemost Thromb. 2005; 16(4): 301–10.</mixed-citation></citation-alternatives></ref><ref id="cit4"><label>4</label><citation-alternatives><mixed-citation xml:lang="ru">Butwick A.J., Goodnough L.T. Transfusion and coagulation management in major obstetric hemorrhage. Curr Opin Anaesthesiol. 2015; 28(3): 275–84.</mixed-citation><mixed-citation xml:lang="en">Butwick A.J., Goodnough L.T. Transfusion and coagulation management in major obstetric hemorrhage. Curr Opin Anaesthesiol. 2015; 28 (3): 275–84.</mixed-citation></citation-alternatives></ref><ref id="cit5"><label>5</label><citation-alternatives><mixed-citation xml:lang="ru">Allard S., Green L., Hunt B.J. How we manage the haematological aspects of major obstetric haemorrhage. Br J Haematol. 2014; 164(2): 177–88.</mixed-citation><mixed-citation xml:lang="en">Allard S., Green L., Hunt B.J. How we manage the haematological aspects of major obstetric haemorrhage. Br J Haematol. 2014; 164(2): 177–88.</mixed-citation></citation-alternatives></ref><ref id="cit6"><label>6</label><citation-alternatives><mixed-citation xml:lang="ru">Guasch E., Gilsanz F. Massive obstetric hemorrhage: Current approach to management. Med Intensiva. 2016; 40(5): 298–310.</mixed-citation><mixed-citation xml:lang="en">Guasch E., Gilsanz F. Massive obstetric hemorrhage: Current approach to management. Med Intensiva. 2016; 40(5): 298–310.</mixed-citation></citation-alternatives></ref><ref id="cit7"><label>7</label><citation-alternatives><mixed-citation xml:lang="ru">Kander T., Larsson A., Taune V. et al. Assessment of Haemostasis in Disseminated Intravascular Coagulation by Use of Point-of-Care Assays and Routine Coagulation Tests, in Critically Ill Patients; A Prospective Observational Study. PloS One. 2016; 11(3): e0151202.</mixed-citation><mixed-citation xml:lang="en">Kander T., Larsson A., Taune V. et al. Assessment of Haemostasis in Disseminated Intravascular Coagulation by Use of Point-of-Care Assays and Routine Coagulation Tests, in Critically Ill Patients; A Prospective Observational Study. PloS One. 2016; 11(3): e0151202.</mixed-citation></citation-alternatives></ref><ref id="cit8"><label>8</label><citation-alternatives><mixed-citation xml:lang="ru">Wikkelsø A., Wetterslev J., Møller A.M., Afshari A. Thromboelastography (TEG) or rotational thromboelastometry (ROTEM) to monitor haemostatic treatment in bleeding patients: a systematic review with meta-analysis and trial sequential analysis. Anaesthesia. 2017; 72(4): 519–31. DOI: 10.1111/anae.13765</mixed-citation><mixed-citation xml:lang="en">Wikkelsø A., Wetterslev J., Møller A.M., Afshari A. Thromboelastography (TEG) or rotational thromboelastometry (ROTEM) to monitor haemostatic treatment in bleeding patients: a systematic review with meta-analysis and trial sequential analysis. Anaesthesia. 2017; 72(4): 519–31. DOI: 10.1111/anae.13765</mixed-citation></citation-alternatives></ref><ref id="cit9"><label>9</label><citation-alternatives><mixed-citation xml:lang="ru">Meybohm P., Zacharowski K., Weber C.F. Point-of-care coagulation management in intensive care medicine. Crit care. 2013; 17(2): 218.</mixed-citation><mixed-citation xml:lang="en">Meybohm P., Zacharowski K., Weber C.F. Point-of-care coagulation management in intensive care medicine. Crit care. 2013; 17(2): 218.</mixed-citation></citation-alternatives></ref><ref id="cit10"><label>10</label><citation-alternatives><mixed-citation xml:lang="ru">Curry N.S., Davenport R., Pavord S. et al. The use of viscoelastic haemostatic assays in the management of major bleeding. Br J Haematol. 2018; 182(6): 789–806.</mixed-citation><mixed-citation xml:lang="en">Curry N.S., Davenport R., Pavord S. et al. The use of viscoelastic haemostatic assays in the management of major bleeding. Br J Haematol. 2018;182(6): 789–806.</mixed-citation></citation-alternatives></ref><ref id="cit11"><label>11</label><citation-alternatives><mixed-citation xml:lang="ru">Korpallova B., Samos M., Bolek T. et al. Role of Thromboelastography and Rotational Thromboelastometry in the Management of Cardiovascular Diseases. Clin Appl Thromb Hemos. 2018; 24(8): 1199–207.</mixed-citation><mixed-citation xml:lang="en">Korpallova B., Samos M., Bolek T. et al. Role of Thromboelastography and Rotational Thromboelastometry in the Management of Cardiovascular Diseases. Clin Appl Thromb Hemos. 2018; 24(8): 1199–207.</mixed-citation></citation-alternatives></ref><ref id="cit12"><label>12</label><citation-alternatives><mixed-citation xml:lang="ru">Zaky A. Thromboelastometry Versus Rotational Thromboelastography in Cardiac Surgery. Semin Cardiothorac Vasc Anesth. 2017; 21(3): 206–11.</mixed-citation><mixed-citation xml:lang="en">Zaky A. Thromboelastometry Versus Rotational Thromboelastography in Cardiac Surgery. Semin Cardiothorac Vasc Anesth. 2017; 21(3): 206–11.</mixed-citation></citation-alternatives></ref><ref id="cit13"><label>13</label><citation-alternatives><mixed-citation xml:lang="ru">Weber C.F., Görlinger K., Meininger D. et al. Point-of-Care Testing. Anesthesiology. 2012; 117(3): 531–47.</mixed-citation><mixed-citation xml:lang="en">Weber C.F., Görlinger K., Meininger D. et al. Point-of-Care Testing. Anesthesiology. 2012; 117(3): 531–47.</mixed-citation></citation-alternatives></ref><ref id="cit14"><label>14</label><citation-alternatives><mixed-citation xml:lang="ru">Görlinger K., Jambor C., Hanke A.A. et al. Perioperative Coagulation Management and Control of Platelet Transfusion by Point-of-Care Platelet Function Analysis. Transfus Med Hemother. 2007; 34(6): 396–411. DOI: 10.1159/000109642</mixed-citation><mixed-citation xml:lang="en">Görlinger K., Jambor C., Hanke A.A. et al. Perioperative Coagulation Management and Control of Platelet Transfusion by Point-of-Care Platelet Function Analysis. Transfus Med Hemother. 2007; 34(6): 396–411. DOI: 10.1159/000109642</mixed-citation></citation-alternatives></ref><ref id="cit15"><label>15</label><citation-alternatives><mixed-citation xml:lang="ru">Rugeri L., Levrat A., David J.S. et al. Diagnosis of early coagulation abnormalities in trauma patients by rotation thrombelastography. J Thromb Haemost. 2007; 5(2): 289–95.</mixed-citation><mixed-citation xml:lang="en">Rugeri L., Levrat A., David J.S. et al. Diagnosis of early coagulation abnormalities in trauma patients by rotation thrombelastography. J Thromb Haemost. 2007; 5(2): 289–95.</mixed-citation></citation-alternatives></ref><ref id="cit16"><label>16</label><citation-alternatives><mixed-citation xml:lang="ru">Rizoli S., Min A., Perez Sanchez A. et al. In Trauma, Conventional ROTEM and TEG Results Are Not Interchangeable But Are Similar in Clinical Applicability. Mil Med. 2016; 181(5S): 117–26.</mixed-citation><mixed-citation xml:lang="en">Rizoli S., Min A., Perez Sanchez A. et al. In Trauma, Conventional ROTEM and TEG Results Are Not Interchangeable But Are Similar in Clinical Applicability. Mil Med. 2016; 181(5S): 117–26.</mixed-citation></citation-alternatives></ref><ref id="cit17"><label>17</label><citation-alternatives><mixed-citation xml:lang="ru">da Luz L.T., Nascimento B., Rizoli S. Thrombelastography (TEG®): practical considerations on its clinical use in trauma resuscitation. Scand J Trauma, Resusc Emerg Med. 2013; 21(1): 29.</mixed-citation><mixed-citation xml:lang="en">da Luz L.T., Nascimento B., Rizoli S. Thrombelastography (TEG®): practical considerations on its clinical use in trauma resuscitation. Scand J Trauma, Resusc Emerg Med. 2013; 21(1): 29.</mixed-citation></citation-alternatives></ref><ref id="cit18"><label>18</label><citation-alternatives><mixed-citation xml:lang="ru">Gonzalez E., Moore E.E., Moore H.B. Management of Trauma-Induced Coagulopathy with Thrombelastography. Crit Care Clin. 2017; 33(1): 119–34.</mixed-citation><mixed-citation xml:lang="en">Gonzalez E., Moore E.E., Moore H.B. Management of Trauma-Induced Coagulopathy with Thrombelastography. Crit Care Clin. 2017; 33(1): 119–34.</mixed-citation></citation-alternatives></ref><ref id="cit19"><label>19</label><citation-alternatives><mixed-citation xml:lang="ru">Abuelkasem E., Lu S., Tanaka K. et al. Comparison between thrombelastography and thromboelastometry in hyperfi brinolysis detection during adult liver transplantation. Br J Anaesth. 2016; 116(4): 507–12.</mixed-citation><mixed-citation xml:lang="en">Abuelkasem E., Lu S., Tanaka K. et al. Comparison between thrombelastography and thromboelastometry in hyperfi brinolysis detection during adult liver transplantation. Br J Anaesth. 2016; 116(4): 507–12.</mixed-citation></citation-alternatives></ref><ref id="cit20"><label>20</label><citation-alternatives><mixed-citation xml:lang="ru">Roullet S., Labrouche S., Mouton C. et al. Lysis Timer: a new sensitive tool to diagnose hyperfi brinolysis in liver transplantation. J Clin Pathol. 2019; 72(1): 58–65.</mixed-citation><mixed-citation xml:lang="en">Roullet S., Labrouche S., Mouton C. et al. Lysis Timer: a new sensitive tool to diagnose hyperfi brinolysis in liver transplantation. J Clin Pathol. 2019; 72 (1): 58–65.</mixed-citation></citation-alternatives></ref><ref id="cit21"><label>21</label><citation-alternatives><mixed-citation xml:lang="ru">Приказ Министерства здравоохранения и социального развития РФ от 13 апреля 2011 г. № 315н «Об утверждении Порядка оказания анестезиолого-реанимационной помощи взрослому населению».</mixed-citation><mixed-citation xml:lang="en">The Order of the Ministry of Health and Social Development of the Russian Federation of April 13, 2011 № 315n “On approval of the provision of anesthesia and resuscitation care to the adult population” (In Russian).</mixed-citation></citation-alternatives></ref><ref id="cit22"><label>22</label><citation-alternatives><mixed-citation xml:lang="ru">Баркаган З.С., Момот А.П. Диагностика и контролируемая терапия нарушений гемостаза. 3-е изд. М.: Ньюдиамед, 2008; 292.</mixed-citation><mixed-citation xml:lang="en">Barkagan Z.S., Momot A.P. Diagnostics and management of hemostasis disorders. 3d edition. Moscow. Newdiamed; 2008; 292:25, 103–13 (In Russian).</mixed-citation></citation-alternatives></ref><ref id="cit23"><label>23</label><citation-alternatives><mixed-citation xml:lang="ru">Зозуля Н.И., Свирин П.В. Клинические рекомендации по диагностике и лечению редких коагулопатий: Наследственный дефицит факторов свертывания крови II, VII, X. НГО. 2014; 35: 19.</mixed-citation><mixed-citation xml:lang="en">Zozulja N.I, Svirin P.V. Clinical practice guidelines for diagnostics and management of rare coagulopathy: inherited coagulation factors defi ciencies. National hematology society. 2014; 35: 19 (In Russian).</mixed-citation></citation-alternatives></ref><ref id="cit24"><label>24</label><citation-alternatives><mixed-citation xml:lang="ru">Peyvandi F., Palla R., Menegatti M. et al. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: Results from the European Network of Rare Bleeding Disorders. J Thromb Haemost. 2012; 10(4): 615–21.</mixed-citation><mixed-citation xml:lang="en">Peyvandi F., Palla R., Menegatti M. et al. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: Results from the European Network of Rare Bleeding Disorders. J Thromb Haemost. 2012; 10(4): 615–21.</mixed-citation></citation-alternatives></ref><ref id="cit25"><label>25</label><citation-alternatives><mixed-citation xml:lang="ru">Acharya S.S., Coughlin A., Dimichele D.M., North American Rare Bleeding Disorder Stud Group. Rare Bleeding Disorder Registry: defi ciencies of factors II, V, VII, X, XIII, fi brinogen and dysfi brinogenemias. J Thromb Haemost. 2004; 2: 248–56.</mixed-citation><mixed-citation xml:lang="en">Acharya S.S., Coughlin A., Dimichele D.M., North American Rare Bleeding Disorder Stud Group. Rare Bleeding Disorder Registry: defi ciencies of factors II, V, VII, X, XIII, fi brinogen and dysfi brinogenemias. J Thromb Haemost. 2004; 2: 248–56.</mixed-citation></citation-alternatives></ref><ref id="cit26"><label>26</label><citation-alternatives><mixed-citation xml:lang="ru">Peyvandi F., Spreafi co M. National and international registries of rare bleeding disorders. Blood Transfus. 2008; 6(Suppl. 2): 45–8.</mixed-citation><mixed-citation xml:lang="en">Peyvandi F., Spreafi co M. National and international registries of rare bleeding disorders. Blood Transfus. 2008; 6(Suppl. 2): 45–8.</mixed-citation></citation-alternatives></ref><ref id="cit27"><label>27</label><citation-alternatives><mixed-citation xml:lang="ru">Rezende S.M., Rodrigues S.H.L., Brito K.N.P. et al. Evaluation of a webbased registry of inherited bleeding disorders: A descriptive study of the Brazilian experience with HEMOVIDAweb Coagulopatias. Orphanet J Rare Dis. 2017; 12(1): 1–7.</mixed-citation><mixed-citation xml:lang="en">Rezende S.M., Rodrigues S.H.L., Brito K.N.P. et al. Evaluation of a webbased registry of inherited bleeding disorders: A descriptive study of the Brazilian experience with HEMOVIDAweb Coagulopatias. Orphanet J Rare Dis. 2017; 12(1): 1–7.</mixed-citation></citation-alternatives></ref><ref id="cit28"><label>28</label><citation-alternatives><mixed-citation xml:lang="ru">Abbonizio F., Hassan H.J., Riccioni R. et al. New data from the Italian National Register of Congenital Coagulopathies, 2016 Annual Survey. Blood Transfus. 2019; 1–8.</mixed-citation><mixed-citation xml:lang="en">Abbonizio F., Hassan H.J., Riccioni R. et al. New data from the Italian National Register of Congenital Coagulopathies, 2016 Annual Survey. Blood Transfus. 2019; 1–8.</mixed-citation></citation-alternatives></ref><ref id="cit29"><label>29</label><citation-alternatives><mixed-citation xml:lang="ru">Мамаев А.Н. Коагулопатии. М.: ГЭОТАР-Медиа, 2012; 260.</mixed-citation><mixed-citation xml:lang="en">Mamaev AN. Coagulopathies. Moscow: GEHOTAR-Media, 2012; 260 (In Russian).</mixed-citation></citation-alternatives></ref><ref id="cit30"><label>30</label><citation-alternatives><mixed-citation xml:lang="ru">Palla R., Peyvandi F., Shapiro A.D. Rare bleeding disorders: Diagnosis and treatment. Blood. 2015; 125(13): 2052–61.</mixed-citation><mixed-citation xml:lang="en">Palla R., Peyvandi F., Shapiro A.D. Rare bleeding disorders: Diagnosis and treatment. Blood. 2015; 125(13): 2052–61.</mixed-citation></citation-alternatives></ref><ref id="cit31"><label>31</label><citation-alternatives><mixed-citation xml:lang="ru">Herrmann F.H., Wulff K., Auberger K. et al. Molecular biology and clinical manifestation of hereditary factor VII defi ciency. Semin Thromb Hemost. 2000; 26(4): 393–400.</mixed-citation><mixed-citation xml:lang="en">Herrmann F.H., Wulff K., Auberger K. et al. Molecular biology and clinical manifestation of hereditary factor VII defi ciency. Semin Thromb Hemost. 2000; 26(4): 393–400.</mixed-citation></citation-alternatives></ref><ref id="cit32"><label>32</label><citation-alternatives><mixed-citation xml:lang="ru">Martinowitz U., Michaelson M. Guidelines for the use of recombinant activated factor VII (rFVIIa) in uncontrolled bleeding: a report by the Israeli multidisciplinary rFVIIa task force. J Thromb Haemost. 2005; 3(4): 640–8.</mixed-citation><mixed-citation xml:lang="en">Martinowitz U., Michaelson M. Guidelines for the use of recombinant activated factor VII (rFVIIa) in uncontrolled bleeding: a report by the Israeli multidisciplinary rFVIIa task force. J Thromb Haemost. 2005; 3(4): 640–8.</mixed-citation></citation-alternatives></ref><ref id="cit33"><label>33</label><citation-alternatives><mixed-citation xml:lang="ru">Simão F., Feener E.P. The effects of the contact activation system on hemorrhage. Front Med. 2017; 4: 1–10.</mixed-citation><mixed-citation xml:lang="en">Simão F., Feener E.P. The effects of the contact activation system on hemorrhage. Front Med. 2017; 4: 1–10.</mixed-citation></citation-alternatives></ref><ref id="cit34"><label>34</label><citation-alternatives><mixed-citation xml:lang="ru">Bachler M., Niederwanger C., Hell T. et al. Infl uence of factor XII defi ciency on activated partial thromboplastin time (aPTT) in critically ill patients. J Thromb Thrombolysis. 2019. DOI: 10.1007/s11239-019-01879-w</mixed-citation><mixed-citation xml:lang="en">Bachler M., Niederwanger C., Hell T. et al. Infl uence of factor XII defi ciency on activated partial thromboplastin time (aPTT) in critically ill patients. J Thromb Thrombolysis. 2019. DOI: 10.1007/s11239-019-01879-w</mixed-citation></citation-alternatives></ref><ref id="cit35"><label>35</label><citation-alternatives><mixed-citation xml:lang="ru">Долгов В., Свирин ПВ. Лабораторная диагностика нарушений гемостаза. М.–Тверь: Триада; 2005: 227.</mixed-citation><mixed-citation xml:lang="en">Dolgov V., Svirin P.V. Laboratory diagnostics of hemostasis disorders. Moscow — Tver’: Triada, 2005; 227 (In Russian).</mixed-citation></citation-alternatives></ref><ref id="cit36"><label>36</label><citation-alternatives><mixed-citation xml:lang="ru">Langdell R., Wagner R., Brinkhous K. Effect of antihemophilic factor on onestage clotting tests; a presumptive test for hemophilia and a simple one-stage antihemophilic factor assy procedure. J Lab Clin Med. 1953; 41(4): 637–47.</mixed-citation><mixed-citation xml:lang="en">Langdell R., Wagner R., Brinkhous K. Effect of antihemophilic factor on onestage clotting tests; a presumptive test for hemophilia and a simple one-stage antihemophilic factor assy procedure. J Lab Clin Med. 1953; 41(4):637–47.</mixed-citation></citation-alternatives></ref><ref id="cit37"><label>37</label><citation-alternatives><mixed-citation xml:lang="ru">Biggs R., Eveling J., Richards G. The Assay of Antihaemophilic-Globulin Activity. Br J Haematol. 1955; 1(1): 20–34.</mixed-citation><mixed-citation xml:lang="en">Biggs R., Eveling J., Richards G. The Assay of Antihaemophilic-Globulin Activity. Br J Haematol. 1955; 1(1): 20–34.</mixed-citation></citation-alternatives></ref><ref id="cit38"><label>38</label><citation-alternatives><mixed-citation xml:lang="ru">Лубнин А.Ю., Коновалов А.Н., Ласунин Н.В. и др. Тяжелые послеоперационные интракраниальные геморрагические осложнения у нейрохирургического больного с не диагностированной до операции болезнью Виллебранда (клиническое наблюдение и обзор литературы). Вопросы нейрохирургии» имени Н.Н. Бурденко. 2018; 82(3): 56–65. DOI: 10.17116/neiro201882356</mixed-citation><mixed-citation xml:lang="en">Lubnin A.Y., Konovalov A.N., Lasunin N.V. et al. Severe postoperative intracranial hemorrhagic complications in a neurosurgical patient with von Willebrand disease not diagnosed before surgery (a case report and literature review. Vopr Nejrokhirurgii Im NN Burdenko. 2018; 82(3): 56–65 (In Russian). DOI: 10.17116/neiro201882356</mixed-citation></citation-alternatives></ref><ref id="cit39"><label>39</label><citation-alternatives><mixed-citation xml:lang="ru">Rugeri L., Quélin F., Chatard B. et al. Thrombin generation in patients with factor XI defi ciency and clinical bleeding risk. Haemophilia. 2010; 16(5): 771–7.</mixed-citation><mixed-citation xml:lang="en">Rugeri L., Quélin F., Chatard B. et al. Thrombin generation in patients with factor XI defi ciency and clinical bleeding risk. Haemophilia. 2010; 16(5): 771–7.</mixed-citation></citation-alternatives></ref><ref id="cit40"><label>40</label><citation-alternatives><mixed-citation xml:lang="ru">Hemker H., Giansily M., Peyvandi F. et al. The Thrombogram in Rare Inherited Coagulation Disorders: Its Relation to Clinical Bleeding. Thromb Haemost. 2018; 88(10): 576–82.</mixed-citation><mixed-citation xml:lang="en">Hemker H., Giansily M., Peyvandi F. et al. The Thrombogram in Rare Inherited Coagulation Disorders: Its Relation to Clinical Bleeding. Thromb Haemost. 2018; 88(10): 576–82.</mixed-citation></citation-alternatives></ref><ref id="cit41"><label>41</label><citation-alternatives><mixed-citation xml:lang="ru">Van Geffen M., Menegatti M., Loof A. et al. Retrospective evaluation of bleeding tendency and simultaneous thrombin and plasmin generation in patients with rare bleeding disorders. Haemophilia. 2012; 18(4): 630–8.</mixed-citation><mixed-citation xml:lang="en">Van Geffen M., Menegatti M., Loof A. et al. Retrospective evaluation of bleeding tendency and simultaneous thrombin and plasmin generation in patients with rare bleeding disorders. Haemophilia. 2012; 18(4): 630–8.</mixed-citation></citation-alternatives></ref><ref id="cit42"><label>42</label><citation-alternatives><mixed-citation xml:lang="ru">Spiezia L., Radu C., Campello E. et al. Whole blood rotation thromboelastometry (ROTEM®) in nine severe factor V defi cient patients and evaluation of the role of intraplatelets factor V. Haemophilia. 2012; 18(3): 463–8.</mixed-citation><mixed-citation xml:lang="en">Spiezia L., Radu C., Campello E. et al. Whole blood rotation thromboelastometry (ROTEM®) in nine severe factor V defi cient patients and evaluation of the role of intraplatelets factor V. Haemophilia. 2012; 18(3): 463–8.</mixed-citation></citation-alternatives></ref><ref id="cit43"><label>43</label><citation-alternatives><mixed-citation xml:lang="ru">Минов А.Ф., Дзядзько А.М., Руммо О.О. Тромбоэластометрические критерии коррекции нарушений гемостаза при трансплантации печени. Анестезиология и реаниматология. 2012; 57(2): 35–41.</mixed-citation><mixed-citation xml:lang="en">Minov A.F., Dzyadzko A.M., Rummo O.O. The tromboelastometric criteria of hemostasis disorders correction during liver transplantation. Anesteziologiya i reanimatologiya. 2012; 57(2): 35–41 (In Russian).</mixed-citation></citation-alternatives></ref><ref id="cit44"><label>44</label><citation-alternatives><mixed-citation xml:lang="ru">Haas T., Cushing M.M., Asmis L.M. Infl uence of Factor V Defi ciency on ROTEM® Clotting Time. Blood. 2014; 124(21): 5039.</mixed-citation><mixed-citation xml:lang="en">Haas T., Cushing M.M., Asmis L.M. Infl uence of Factor V Defi ciency on ROTEM® Clotting Time. Blood. 2014; 124(21): 5039.</mixed-citation></citation-alternatives></ref><ref id="cit45"><label>45</label><citation-alternatives><mixed-citation xml:lang="ru">Chakri G., Yates S.G., Rambally S., Sarode R. Transfusion management of factor V defi ciency: three case reports and review of the literature. Transfusion. 2016; 56(7): 1745–9.</mixed-citation><mixed-citation xml:lang="en">Chakri G., Yates S.G., Rambally S., Sarode R. Transfusion management of factor V defi ciency: three case reports and review of the literature. Transfusion. 2016; 56(7): 1745–9.</mixed-citation></citation-alternatives></ref><ref id="cit46"><label>46</label><citation-alternatives><mixed-citation xml:lang="ru">Tran H.T.T., Tjønnfjord G.E., Holme P.A. Use of thromboelastography and thrombin generation assay to predict clinical phenotype in patients with severe FVII defi ciency. Haemophilia. 2013; 20(1): 141–6.</mixed-citation><mixed-citation xml:lang="en">Tran H.T.T., Tjønnfjord G.E., Holme P.A. Use of thromboelastography and thrombin generation assay to predict clinical phenotype in patients with severe FVII defi ciency. Haemophilia. 2013; 20(1): 141–6.</mixed-citation></citation-alternatives></ref><ref id="cit47"><label>47</label><citation-alternatives><mixed-citation xml:lang="ru">Nogami K. The utility of thromboelastography in inherited and acquired bleeding disorders. Br J Haematol. 2016; 174(4): 503–14.</mixed-citation><mixed-citation xml:lang="en">Nogami K. The utility of thromboelastography in inherited and acquired bleeding disorders. Br J Haematol. 2016; 174(4): 503–14.</mixed-citation></citation-alternatives></ref><ref id="cit48"><label>48</label><citation-alternatives><mixed-citation xml:lang="ru">Dirkmann D., Hanke A.A., Görlinger K., Peters J. Perioperative use of modifi ed thrombelastography in factor XI defi ciency: A helpful method to assess drug effects. Acta Anaesthesiol Scand. 2007; 51(5): 640–3. DOI: 10.1111/j.1399-6576.2007.01284.x</mixed-citation><mixed-citation xml:lang="en">Dirkmann D., Hanke A.A., Görlinger K., Peters J. Perioperative use of modifi ed thrombelastography in factor XI defi ciency: A helpful method to assess drug effects. Acta Anaesthesiol Scand. 2007; 51(5): 640–3. DOI: 10.1111/j.1399-6576.2007.01284.x</mixed-citation></citation-alternatives></ref><ref id="cit49"><label>49</label><citation-alternatives><mixed-citation xml:lang="ru">Riddell A., Abdul-Kadir R., Pollard D. et al. Monitoring low dose recombinant factor VIIa therapy in patients with severe factor XI defi ciency undergoing surgery. Thromb Haemost. 2011; 106(9): 521–7.</mixed-citation><mixed-citation xml:lang="en">Riddell A., Abdul-Kadir R., Pollard D. et al. Monitoring low dose recombinant factor VIIa therapy in patients with severe factor XI defi ciency undergoing surgery. Thromb Haemost. 2011; 106(9): 521–7.</mixed-citation></citation-alternatives></ref><ref id="cit50"><label>50</label><citation-alternatives><mixed-citation xml:lang="ru">Pivalizza E.G. Perioperative use of the Thrombelastograph® in patients with inherited bleeding disorders. J Clin Anesth. 2003; 15(5): 366–70.</mixed-citation><mixed-citation xml:lang="en">Pivalizza E.G. Perioperative use of the Thrombelastograph® in patients with inherited bleeding disorders. J Clin Anesth. 2003; 15(5): 366–70.</mixed-citation></citation-alternatives></ref><ref id="cit51"><label>51</label><citation-alternatives><mixed-citation xml:lang="ru">Pluthero F.G., Ryan C., Williams S. et al. Decreased in vitro thrombin generation and clot stability in human FXII-null blood and plasma. Br J Haematol. 2011; 152(1): 111–2.</mixed-citation><mixed-citation xml:lang="en">Pluthero F.G., Ryan C., Williams S. et al. Decreased in vitro thrombin generation and clot stability in human FXII-null blood and plasma. Br J Haematol. 2011; 152(1): 111–2.</mixed-citation></citation-alternatives></ref><ref id="cit52"><label>52</label><citation-alternatives><mixed-citation xml:lang="ru">Blois S.L., Holowaychuk M.K., Wood R.D. Evaluation of thromboelastography in two factor XII-defi cient cats. JFMS Open Rep. 2015; 1(1): 205511691558502. DOI: 10.1177/2055116915585025</mixed-citation><mixed-citation xml:lang="en">Blois S.L., Holowaychuk M.K., Wood R.D. Evaluation of thromboelastography in two factor XII-defi cient cats. JFMS Open Rep. 2015; 1(1): 205511691558502. DOI: 10.1177/2055116915585025</mixed-citation></citation-alternatives></ref></ref-list><fn-group><fn fn-type="conflict"><p>The authors declare that there are no conflicts of interest present.</p></fn></fn-group></back></article>
