<?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">nodgo</journal-id><journal-title-group><journal-title xml:lang="ru">Российский журнал детской гематологии и онкологии (РЖДГиО)</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Journal of Pediatric Hematology and Oncology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2311-1267</issn><issn pub-type="epub">2413-5496</issn><publisher><publisher-name>LTD “Graphica”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21682/2311-1267-2020-7-3-132-137</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-637</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>КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CLINICAL CASES</subject></subj-group></article-categories><title-group><article-title>Клинический случай успешного применения рекомбинантного активированного VII фактора свертывания у новорожденного с сепсисом</article-title><trans-title-group xml:lang="en"><trans-title>A clinical case of the successful use of recombinant activated VII coagulation factor in a newborn with sepsis</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2191-1087</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Труханова</surname><given-names>И. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Trukhanova</surname><given-names>I. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Инна Георгиевна Труханова, доктор медицинских наук, профессор, заведующая кафедрой анестезиологии, реаниматологии и скорой медицинской помощи, главный внештатный специалист по скорой медицинской помощи Минздрава Самарской области</p><p>443099, Самара, ул. Чапаевская, 89</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Head of the Department of Anesthesiology, Intensive Care and Emergency Medicine, Chief External Expert of Emergency Medicine of the Ministry of Health of the Samara Region</p><p>89 Chapaevskaya St., Samara, 443099</p></bio><email xlink:type="simple">innasmp@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/0000-0003-0625-2172</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кругова</surname><given-names>Л. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Krugova</surname><given-names>L. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кандидат медицинских наук, доцент кафедры анестезиологии, реаниматологии и скорой медицинской помощи, заведующая отделением анестезиологии и реанимации для беременных и рожениц ТГКБ № 2</p><p>443099, Самара, ул. Чапаевская, 89, </p><p>445020, Тольятти, ул. Баныкина, 8</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Associate Professor of the Department of Anesthesiology, Intensive Care and Emergency Medicine, Head of the Department of Anesthesiology and Intensive Care for Pregnant Women TGKB № 2</p><p>89 Chapaevskaya St., Samara, 443099, </p><p>8 Banykina St., Togliatti, 445020</p></bio><email xlink:type="simple">likrugova@yandex.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6326-8878</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кутырева</surname><given-names>Ю. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Kutyreva</surname><given-names>Yu. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кандидат медицинских наук, доцент кафедры анестезиологии, реаниматологии и скорой медицинской помощи, врач анестезиолог-реаниматолог, главный врач ООО «Курорты Поволжья»</p><p>443099, Самара, ул. Чапаевская, 89, </p><p>443051, Самара, ул. Енисейская, 62</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Associate Professor of the Department of Anesthesiology, Intensive Care and Emergency Medicine, Doctor of Anesthesiology and Intensive Care, Chief Physician of Volga Resorts LLC</p><p>89 Chapaevskaya St., Samara, 443099, </p><p>62 Yeniseyskaya St., Samara, 443051</p></bio><email xlink:type="simple">jiul.kutyriova@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБОУ ВО «Самарский государственный медицинский университет» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Samara State Medical University, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФГБОУ ВО «Самарский государственный медицинский университет» Минздрава России;</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Samara State Medical University, Ministry of Health of Russia;&#13;
Togliatti City Clinical Hospital № 2</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>ФГБОУ ВО «Самарский государственный медицинский университет» Минздрава России;</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Samara State Medical University, Ministry of Health of Russia;&#13;
Volga Resorts</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2020</year></pub-date><pub-date pub-type="epub"><day>21</day><month>09</month><year>2020</year></pub-date><volume>7</volume><issue>3</issue><fpage>132</fpage><lpage>137</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Труханова И.Г., Кругова Л.В., Кутырева Ю.Г., 2020</copyright-statement><copyright-year>2020</copyright-year><copyright-holder xml:lang="ru">Труханова И.Г., Кругова Л.В., Кутырева Ю.Г.</copyright-holder><copyright-holder xml:lang="en">Trukhanova I.G., Krugova L.V., Kutyreva Y.G.</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.nodgo.org/jour/article/view/637">https://journal.nodgo.org/jour/article/view/637</self-uri><abstract><p>В последнее время при проведении интенсивной терапии приобретенных коагулопатий у детей и новорожденных важное место занимают синтетические факторы свертывания крови, при этом все чаще используется рекомбинантный VII активированный фактор свертывания крови (rFVIIa). Он инициирует гемостаз в месте повреждения сосудов, формирует комплекс с тканевым фактором, обеспечивает максимальную активацию тромбоцитов. Образовавшийся комплекс стимулирует переход факторов свертывания крови IХ и X в активную форму IХа и Xа, затем фактор Xa приводит к усилению синтеза тромбина и формированию стабильного фибринового сгустка. Изначально rFVIIa был разработан и применялся для лечения кровотечений и проведения хирургических операций у пациентов с наследственной или приобретенной гемофилией и высоким титром ингибитора к факторам свертывания VIII или IX. В настоящее время показания к его применению значительно расширяются, так как он является эффективным при интенсивной терапии других приобретенных коагулопатиий, в том числе при возникновении коагулопатии, обусловленной сепсисом. В данной статье представлен клинический случай успешного применения rFVIIa у новорожденного с сепсисом и нарушениями гемокоагуляции. Описание каждого наблюдения имеет важное значение для накопления опыта и разработки дальнейших алгоритмов лечения новорожденных с сепсисом, сопровождающимся нарушениями гемокоагуляции, так как единая эффективная тактика ведения таких пациентов на сегодняшний день отсутствует.</p></abstract><trans-abstract xml:lang="en"><p>Recently, in the intensive care of acquired coagulopathies in children and newborns, synthetic coagulation factors have occupied an important place, with recombinant VII activated coagulation factor (rFVIIa) being used more often. It initiates hemostasis at the site of vascular damage, forms a complex with tissue factor, and ensures maximum platelet activation. The resulting complex stimulates the transition of blood coagulation factors IX and X to the active form IXa and Xa, then factor Xa leads to increased synthesis thrombin and the formation of a stable fibrin clot. Initially, rFVIIa was developed and used to treat bleeding and surgery in patients with hereditary or acquired hemophilia and a high titer of an inhibitor to coagulation factors VIII or IX. Currently, indications for its use have expanded significantly and it is effective in intensive therapy of other acquired coagulopathy, including the occurrence of coagulopathy due to sepsis. This article presents a clinical case of the successful use of rFVIIa in a newborn with sepsis and hemocogulation disorders. The description of each case is important for the accumulation of experience and the development of further algorithms for the treatment of newborns with sepsis, accompanied by impaired hemocoagulation, since there is currently no single effective management strategy for such patients.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>сепсис</kwd><kwd>нарушение гемокоагуляции</kwd><kwd>рекомбинантный активированный VII фактор</kwd></kwd-group><kwd-group xml:lang="en"><kwd>sepsis</kwd><kwd>impaired blood coagulation</kwd><kwd>recombinant activated factor VII</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">Tan L.N., Mariappa G., Voon H.Y., Suharjono H. Septic miscarriage with toxic shock syndrome and disseminated intravascular coagulation (DIC): The role of surgery, recombinant activated factor VII and intravenous immunoglobulin (IVIG). Med J Malaysia 2017;72(6):380–1. PMID: 22557832.</mixed-citation><mixed-citation xml:lang="en">Tan L.N., Mariappa G., Voon H.Y., Suharjono H. Septic miscarriage with toxic shock syndrome and disseminated intravascular coagulation (DIC): The role of surgery, recombinant activated factor VII and intravenous immunoglobulin (IVIG). Med J Malaysia 2017;72(6):380–1. PMID: 22557832.</mixed-citation></citation-alternatives></ref><ref id="cit2"><label>2</label><citation-alternatives><mixed-citation xml:lang="ru">Madkhali Y., Featherby S., Collier M.E., Maraveyas A., Greenman J., Ettelaie C. The Ratio of Factor VIIa: Tissue Factor Content with in Microvesicles Determines the Diﬀerential Inﬂuence on Endothelial Cells. TH Open 2019;3(2):e132–45. doi: 10.1055/s-0039-1688934.</mixed-citation><mixed-citation xml:lang="en">Madkhali Y., Featherby S., Collier M.E., Maraveyas A., Greenman J., Ettelaie C. The Ratio of Factor VIIa: Tissue Factor Content with in Microvesicles Determines the Diﬀerential Inﬂuence on Endothelial Cells. TH Open 2019;3(2):e132–45. doi: 10.1055/s-0039-1688934.</mixed-citation></citation-alternatives></ref><ref id="cit3"><label>3</label><citation-alternatives><mixed-citation xml:lang="ru">Saracco P., Vitale P., Scolfaro C., Pollio B., Pagliarino M., Timeus F. The coagulopathy in sepsis: signiﬁcance and implications for treatment. Pediatr Rep 2011;3(4):e30. doi: 10.4081/pr.2011.e30.</mixed-citation><mixed-citation xml:lang="en">Saracco P., Vitale P., Scolfaro C., Pollio B., Pagliarino M., Timeus F. The coagulopathy in sepsis: signiﬁcance and implications for treatment. Pediatr Rep 2011;3(4):e30. doi: 10.4081/pr.2011.e30.</mixed-citation></citation-alternatives></ref><ref id="cit4"><label>4</label><citation-alternatives><mixed-citation xml:lang="ru">Шутов С.А., Карагюлян С.Р., Буланов А.Ю., Соркина О.М., Баландина А.Н., Васильев С.А. Опыт применения отечественного рекомбинантного активированного фактора VII при выполнении лапароскопической спленэктомии у пациентки с хронической иммунной тромбоцитопенией. Вопросы гематологии/онкологии и иммунопатологии в педиатрии 2012;11(3):2–7. doi: 615.273.5.03:616.155.294-08:616.411-089.87.</mixed-citation><mixed-citation xml:lang="en">Shutov S.A., Karagyulan S.R., Bulanov A.Yu., Sorkina O.M., Balandina A.N., Vasilyev S.A. Russian recombinant activated factor VII was used in laparoscopic splenectomy in a female patient with chronic immune thrombocytopenia. Voprosy gematologii/onkologii i immunopatologii v pediatrii = Pediatric Hematology/Oncology and Immunopathology 2012;11(3):2–7. (In Russ.). doi: 615.273.5.03:616.155.294-08:616.411-089.87.</mixed-citation></citation-alternatives></ref><ref id="cit5"><label>5</label><citation-alternatives><mixed-citation xml:lang="ru">Hoﬀman M. A cell-based model of coagulation and the role of factor VIIa. Blood Rev 2003;17(Suppl 1):S1–5. doi: 10.1016/S0268-960X(03)90000-2.</mixed-citation><mixed-citation xml:lang="en">Hoﬀman M. A cell-based model of coagulation and the role of factor VIIa. Blood Rev 2003;17(Suppl 1):S1–5. doi: 10.1016/S0268-960X(03)90000-2.</mixed-citation></citation-alternatives></ref><ref id="cit6"><label>6</label><citation-alternatives><mixed-citation xml:lang="ru">Diaz R., Almeida P., Alvarez M., Ferrer G., Hernandez F. LifeThreatening Pulmonary Hemorrhage Responds to Recombinant Factor VIIa: A Case Series in South Florida Hospitals. Cureus 2019;11(11):e6202. doi: 10.7759/cureus.6202.</mixed-citation><mixed-citation xml:lang="en">Diaz R., Almeida P., Alvarez M., Ferrer G., Hernandez F. LifeThreatening Pulmonary Hemorrhage Responds to Recombinant Factor VIIa: A Case Series in South Florida Hospitals. Cureus 2019;11(11):e6202. doi: 10.7759/cureus.6202.</mixed-citation></citation-alternatives></ref><ref id="cit7"><label>7</label><citation-alternatives><mixed-citation xml:lang="ru">Кузник Б.И., Стуров В.Г., Левшин Н.Ю., Максимова О.Г., Кудлай Д.А. Геморрагические и тромботические заболевания и синдромы у детей и подростков: Патогенез, клиника, диагностика, терапия и профилактика. Новосибирск: Наука, 2018. 524 с. ISBN-13(EAN): 9785020190801.</mixed-citation><mixed-citation xml:lang="en">Kuznik B.I., Sturov V.G., Levshin N.Yu., Maksimova O.G., Kudlay D.A. Hemorrhagic and thrombotic diseases and syndromes in children and adolescents: Pathogenesis, clinical picture, diagnosis, therapy and prevention. Novosibirsk: Nauka, 2018. 524 p. ISBN-13(EAN): 9785020190801. (In Russ.).</mixed-citation></citation-alternatives></ref><ref id="cit8"><label>8</label><citation-alternatives><mixed-citation xml:lang="ru">Shiller E., Petrov V., Svirin P., Vdovin V., Bullikh A., Nikitin, E., Kudlay D.A. Long-term prophylaxis with activated recombinant FVII in children with hemophilia A and inhibitor, receiving treatment with ITI protocol. Blood 2016;128(22):4980. doi: 10.1182/blood.V128.22.4980.4980.</mixed-citation><mixed-citation xml:lang="en">Shiller E., Petrov V., Svirin P., Vdovin V., Bullikh A., Nikitin, E., Kudlay D.A. Long-term prophylaxis with activated recombinant FVII in children with hemophilia A and inhibitor, receiving treatment with ITI protocol. Blood 2016;128(22):4980. doi: 10.1182/blood.V128.22.4980.4980.</mixed-citation></citation-alternatives></ref><ref id="cit9"><label>9</label><citation-alternatives><mixed-citation xml:lang="ru">Кудлай Д.А. Разработка и применение отечественных рекомбинантных препаратов факторов свертывания крови VII, VIII, IX у детей с гемофилией А и B. Педиатрия. Журнал им. Г.Н. Сперанского 2019;98(1):9–17. doi: doi.org/10.29296/25877305-2019-09-18.</mixed-citation><mixed-citation xml:lang="en">Kudlay D.A. Development and use of Russian recombinant blood coagulation factor VII, VIII, IX drugs in children with hemophilia A and B. Pediatriya. Zhurnal im. G.N. Speranskogo = Pediatria. Journal named after G.N. Speransky 2019;98(1):9–17. (In Russ.). doi: doi.org/10.29296/25877305-2019-09-18.</mixed-citation></citation-alternatives></ref><ref id="cit10"><label>10</label><citation-alternatives><mixed-citation xml:lang="ru">Byskov K., Le Gall S.M., Thiede B., Camerer E., Kanse S.M. Protease activated receptors (PAR)-1 and -2 mediate cellular eﬀects of factor VII activating protease. (FSAP) FASEB J 2020;34(1):1079–90. doi: 10.1096/fj.201801986RR.</mixed-citation><mixed-citation xml:lang="en">Byskov K., Le Gall S.M., Thiede B., Camerer E., Kanse S.M. Protease activated receptors (PAR)-1 and -2 mediate cellular eﬀects of factor VII activating protease. (FSAP) FASEB J 2020;34(1):1079–90. doi: 10.1096/fj.201801986RR.</mixed-citation></citation-alternatives></ref><ref id="cit11"><label>11</label><citation-alternatives><mixed-citation xml:lang="ru">Enoksson M., Martin E.J., Holmberg H., Jensen M.S., KjelgaardHansen M., Egebjerg T., Buchardt J., Krogh T.N., Demuth H., Sanfridson A., Hilden I., Kjalke M., Brophy D.F. Enhanced potency of recombinant factor VIIa with increased aﬃnity to activated platelets. J Thromb Haemost 2020;18(1):104–13. doi: 10.1111/jth.14644.</mixed-citation><mixed-citation xml:lang="en">Enoksson M., Martin E.J., Holmberg H., Jensen M.S., KjelgaardHansen M., Egebjerg T., Buchardt J., Krogh T.N., Demuth H., Sanfridson A., Hilden I., Kjalke M., Brophy D.F. Enhanced potency of recombinant factor VIIa with increased aﬃnity to activated platelets. J Thromb Haemost 2020;18(1):104–13. doi: 10.1111/jth.14644.</mixed-citation></citation-alternatives></ref><ref id="cit12"><label>12</label><citation-alternatives><mixed-citation xml:lang="ru">Трекова Н.А., Гуськов Д.А., Гончарова А.В., Аксельрод Б.А. Эффективность отечественного рекомбинантного активированного фактора VII для лечения кровотечений и нормализации гемостаза при операциях на грудном отделе аорты. Вестник анестезиологии и реаниматологии 2019;16(3):25–31. doi: 10.21292/2078-5658-2019-16-3-25-31.</mixed-citation><mixed-citation xml:lang="en">Trekova N.A., Guskov D.A., Goncharova A.V., Akselrod B.A. The eﬃcacy of recombinant activated factor VII (coagil VII) for treatment haemorrhage and haemostasis disordes during thoracic aorta operations. Vestnik anesteziologii i reanimatologii = Messenger of Anesthesiology and Resuscitation 2019;16(3):25–31. (In Russ.). doi: 10.21292/2078-5658-2019-16-3-25-31.</mixed-citation></citation-alternatives></ref><ref id="cit13"><label>13</label><citation-alternatives><mixed-citation xml:lang="ru">Gorkom B.L., Holme P.A., Joch C., Rogosch T., Feussner A., McKeand W., Roberts J., vanHeerde W. Pharmacokinetics and pharmacodynamics of a recombinant fusion protein linking activated coagulation factor VII with human albumin (rVIIa-FP) in patients with congenital FVII deﬁciency. Hematology 2020;25(1):17–25. doi: 10.1080/16078454.2019.1700329.</mixed-citation><mixed-citation xml:lang="en">Gorkom B.L., Holme P.A., Joch C., Rogosch T., Feussner A., McKeand W., Roberts J., vanHeerde W. Pharmacokinetics and pharmacodynamics of a recombinant fusion protein linking activated coagulation factor VII with human albumin (rVIIa-FP) in patients with congenital FVII deﬁciency. Hematology 2020;25(1):17–25. doi: 10.1080/16078454.2019.1700329.</mixed-citation></citation-alternatives></ref><ref id="cit14"><label>14</label><citation-alternatives><mixed-citation xml:lang="ru">Нехаев И.В., Приходченко А.О., Жужгинова О.В., Ломидзе С.В., Сытов А.В. Рекомбинантный VIIA фактор в интенсивной терапии. Гематология и трансфузиология 2015;2:32–9. doi: 615.273.5.03:616-006.04-089.</mixed-citation><mixed-citation xml:lang="en">Nekhaev I.V., Prikhodchenko A.O., Zhuzhginova O.V., Lomidze S.V., Sytov A.V. Recombinant factor VIIA in intensive care. Gematologiya i transfuziologiya = Hematology and Transfusiology 2015;2:32–9. (In Russ.). doi: 615.273.5.03:616-006.04-089.</mixed-citation></citation-alternatives></ref><ref id="cit15"><label>15</label><citation-alternatives><mixed-citation xml:lang="ru">Андреева Т.А., Зоренко В.Ю., Кудлай Д.А., Лавриченко И.А., Константинова В.Н., Залепухина О.Э., Мишин Г.В., Климова Н.И., Ким А.В., Шустер А.М., Борозинец А.Ю., Казаров А.А.,Жиляева М.В. Исследование иммуногенности российского рекомбинантного активированного фактора свертывания VII у больных гемофилией А, осложненной развитием ингибиторов. Педиатрия. Журнал им. Г.Н. Сперанского 2020;99(2):206–15. doi: 10.21682/2311-1267-2020-7-4-55-57.</mixed-citation><mixed-citation xml:lang="en">Andreeva T.A., Zorenko V.Yu., Kudlay D.A., Lavrichenko I.A., Konstantinova V.N., Zalepukhina O.E., Mishin G.V., Klimova N.I., Kim A V.V., Shuster A.M., Borozinets A.Yu., Kazarov A.A., Zhilyaeva M.V. Immunogenicity study of the russian recombinant activated coagulation factor VII in hemophilia A patients with inhibitors. Pediatriya. Zhurnal im. G.N. Speranskogo = Pediatria. Journal named after G.N. Speransky 2020;99(2):206–15. (In Russ.). doi: 10.21682/2311-1267-2020-7-4-55-57.</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>
