<?xml version="1.0" encoding="UTF-8"?>
<!DOCTYPE root>
<article 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" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Russian Journal of Child Neurology</journal-id><journal-title-group><journal-title xml:lang="en">Russian Journal of Child Neurology</journal-title><trans-title-group xml:lang="ru"><trans-title>Русский журнал детской неврологии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2073-8803</issn><issn publication-format="electronic">2412-9178</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">385</article-id><article-id pub-id-type="doi">10.17650/2073-8803-2021-16-4-42-48</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>ORIGINAL REPORTS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ СТАТЬИ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Diagnosis and treatment of brain compression in children with hemostasis disorders</article-title><trans-title-group xml:lang="ru"><trans-title>Особенности диагностики и лечения сдавления мозга при патологии системы гемостаза у детей раннего возраста</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7061-6300</contrib-id><name-alternatives><name xml:lang="en"><surname>Lagunavichene</surname><given-names>M. L.</given-names></name><name xml:lang="ru"><surname>Лагунавичене</surname><given-names>М. Л.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><bold>Margarita Lvovna Lagunavichene</bold></p><p><italic>664007, Irkutsk, Sovetskaya St., 57</italic></p></bio><bio xml:lang="ru"><p><bold>Маргарита Львовна Лагунавичене</bold></p><p>664007 <italic>Иркутск, ул. Советская, 57</italic></p></bio><email>lagr93@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Larionov</surname><given-names>S. N.</given-names></name><name xml:lang="ru"><surname>Ларионов</surname><given-names>С. Н.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>664007, Irkutsk, Sovetskaya St., 57</italic></p></bio><bio xml:lang="ru"><p><italic>664007 Иркутск, ул. Советская, 57</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Aleksandrov</surname><given-names>Y. A.</given-names></name><name xml:lang="ru"><surname>Александров</surname><given-names>Ю. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>664007, Irkutsk, Sovetskaya St., 57</italic></p></bio><bio xml:lang="ru"><p><italic>664007 Иркутск, ул. Советская, 57</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Livadarov</surname><given-names>A. V.</given-names></name><name xml:lang="ru"><surname>Ливадаров</surname><given-names>А. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>664007, Irkutsk, Sovetskaya St., 57</italic></p></bio><bio xml:lang="ru"><p><italic>664007 Иркутск, ул. Советская, 57</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Gruzin</surname><given-names>P. G.</given-names></name><name xml:lang="ru"><surname>Грузин</surname><given-names>П. Г.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>664007, Irkutsk, Sovetskaya St., 57</italic></p></bio><bio xml:lang="ru"><p><italic>664007 Иркутск, ул. Советская, 57</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Rudakova</surname><given-names>A. V.</given-names></name><name xml:lang="ru"><surname>Рудакова</surname><given-names>А. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>664007, Irkutsk, Sovetskaya St., 57</italic></p></bio><bio xml:lang="ru"><p><italic>664007 Иркутск, ул. Советская, 57</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Osipova</surname><given-names>E. G.</given-names></name><name xml:lang="ru"><surname>Осипова</surname><given-names>Е. Г.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>664007, Irkutsk, Sovetskaya St., 57</italic></p></bio><bio xml:lang="ru"><p><italic>664007, Иркутск, ул. Советская, 57</italic></p></bio><xref ref-type="aff" rid="aff2"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Department of Neurosurgery, Ivano-Matreninskaya Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Нейрохирургическое отделение ОГАУЗ «Городская Ивано-Матренинская детская клиническая больница»</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">Department of Pathology and Premature Infants, Ivano-Matreninskaya Children’s Clinical Hospital</institution></aff><aff><institution xml:lang="ru">Отделение патологии и недоношенных детей ОГАУЗ «Городская Ивано-Матренинская детская клиническая больница»</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2021-12-15" publication-format="electronic"><day>15</day><month>12</month><year>2021</year></pub-date><volume>16</volume><issue>4</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>42</fpage><lpage>48</lpage><history><date date-type="received" iso-8601-date="2022-01-31"><day>31</day><month>01</month><year>2022</year></date><date date-type="accepted" iso-8601-date="2022-01-31"><day>31</day><month>01</month><year>2022</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2021, ABV-Press</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2021, АБВ-пресс</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="en">ABV-Press</copyright-holder><copyright-holder xml:lang="ru">АБВ-пресс</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://rjdn.abvpress.ru/jour/about/editorialPolicies</ali:license_ref></license></permissions><self-uri xlink:href="https://rjdn.abvpress.ru/jour/article/view/385">https://rjdn.abvpress.ru/jour/article/view/385</self-uri><abstract xml:lang="en"><p>We describe the diagnosis of and treatment for brain compression on background of hemostasis disorders in 6 out of 37 patients with hemorrhagic strokes treated in the Ivano-Matreninskaya Children’s Clinical Hospital in 2017–2018. Laboratory examination revealed factor VIII deficiency (hemophilia A) in 3 patients, factor XIII deficiency in one patient; 2 children were carriers of thrombophilia genetic polymorphisms. The age of study participants varied between 15 days and 2 years; all of them were males. They have undergone neurological examination, laboratory testing (hemostasis), neurosonography, multislice computed tomography, and magnetic resonance imaging. Conservative therapy with quarantined fresh frozen plasma (15–25 mL/kg) was used to correct coagulopathy and stop bleeding. Two patients had surgeries: decompressive craniectomy and drainage and endoscopic removal of hematoma. Children that were operated on received transfusion of fresh frozen plasma and red blood cells. Both of them had regression of neurological symptoms and restoration of consciousness in the postoperative period. None of the patients died. In this article, we discuss diagnostics and strategy of conservative and surgical treatment for children with brain compression and coagulopathies.</p></abstract><trans-abstract xml:lang="ru"><p>Представлены результаты диагностики и лечения сдавления мозга на фоне патологии системы гемостаза у 6 из 37 пациентов с геморрагическими инсультами, находившихся на лечении в ОГАУЗ «Городская Ивано-Матренинская детская клиническая больница» в 2017–2018 гг. Лабораторная диагностика выявила у 3 пациентов дефицит компонентов VIII фактора свертываемости (гемофилия типа А), у 1 пациента – дефицит XIII фактора свертываемости, 2 детей явились носителями генетических полиморфизмов тромбофилии. Возраст детей варьировал от 15 дней жизни до 2 лет, все пациенты мужского пола. Комплекс диагностических мероприятий включал неврологический осмотр, лабораторную диагностику системы гемостаза, интроскопические методы исследования: нейросонографию, мультиспиральную компьютерную и магнитно-резонансную томографию. С целью коррекции коагулопатии и остановки кровотечения проводилась консервативная терапия с использованием карантинизированной свежезамороженной плазмы в дозе 15–25 мл/кг. В 2 случаях проводилось хирургическое лечение: в одном случае – декомпрессивная краниотомия, в другом – дренирование и эндоскопическое удаление гематомы. Оперативные вмешательства выполнялись на фоне переливания свежезамороженной плазмы и эритроцитарной массы. В послеоперационном периоде отмечены регресс неврологической симптоматики, восстановление сознания. Летальных исходов не зарегистрировано. Обсуждаются особенности диагностики, тактики консервативного и хирургического лечения детей со сдавлением мозга при коагулопатиях. </p></trans-abstract><kwd-group xml:lang="en"><kwd>coagulopathy</kwd><kwd>blood clotting disorder</kwd><kwd>brain compression</kwd><kwd>fresh frozen plasma</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>коагулопатия</kwd><kwd>нарушение свертываемости крови</kwd><kwd>сдавление мозга</kwd><kwd>свежезамороженная плазма</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">Abdulin D.I., Andreeva E.N., Voevodin S.M. et al. Intraventricular hemorrhages and posthemorrhagic hydrocephalus in newborns. Principles of medical care. Moscow, 2014. Pp. 11–15. (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Абдулин Д.И., Андреева Е.Н., Воеводин С.М. и др. Внутрижелудочковые кровоизлияния, постгеморрагическая гидроцефалия у новорожденных детей. Принципы оказания медицинской помощи. М.: Москва, 2014. C. 11–15.</mixed-citation></citation-alternatives></ref><ref id="B2"><label>2.</label><citation-alternatives><mixed-citation xml:lang="en">Guzeva V.I., Okhrim I.V., Maksimova N.E. et al. Acute period of traumatic brain injury in children with hemophilia. Pediatr = Pediatrician 2017;8(3): 158–63. (In Russ.). DOI:10.17816/PED83158-163.</mixed-citation><mixed-citation xml:lang="ru">Гузева В.И., Охрим И.В., Максимова Н.Е. и др. Особенности острого периода черепно-мозговой травмы у детей с гемофилией. Педиатр 2017;8(3):158–63. DOI:10.17816/PED83158-163.</mixed-citation></citation-alternatives></ref><ref id="B3"><label>3.</label><citation-alternatives><mixed-citation xml:lang="en">Livshits M.I., Popov V.E., Koltunov I.E. et al. Intracranial hemorrhages in children with hemophilia. Rossiyskiy zhurnal detskoy gematologii i onkologii = Russian Journal of Pediatric Hematology and Oncology 2015;2(1):49–53. (In Russ.). DOI:10.17650/2311-1267-2015-1-49-53.</mixed-citation><mixed-citation xml:lang="ru">Лившиц М.И., Попов В.Е., Колтунов И.Е. и др. Внутричерепные кровоизлияния у детей с гемофилией. Российский журнал детской гематологии и онкологии 2015;2(1):49–53. DOI:10.17650/2311-1267-2015-1-49-53.</mixed-citation></citation-alternatives></ref><ref id="B4"><label>4.</label><citation-alternatives><mixed-citation xml:lang="en">Petrukhin A.S., Bobylova M.Yu., Mikhaylova S.V. Etiology of stroke in children. Zhurnal nevrologii i psikhiatrii = Journal of Neurology and Psychiatry 2015;115(3–2):64–74. (In Russ.). DOI:10.17116/jnevro20151153264-74.</mixed-citation><mixed-citation xml:lang="ru">Петрухин А.С., Бобылова М.Ю., Михайлова С.В. Этиология инсульта у детей. Журнал неврологии и психиатрии 2015;115(3–2):64–74. DOI:10.17116/jnevro20151153264-74.</mixed-citation></citation-alternatives></ref><ref id="B5"><label>5.</label><citation-alternatives><mixed-citation xml:lang="en">Popov V.E., Koltunov I.E., Livshits M.I. et al. Hemorrhages in the central nervous system in children with hemophilia. Neyrokhirurgiya i nevrologiya detskogo vozrasta = Pediatric Neurosurgery and Neurology 2017;51(1):41–9. (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Попов В.Е., Колтунов И.Е., Лившиц М.И. и др. Кровоизлияния в структуры центральной нервной системы у детей, больных гемофилией. Нейрохирургия и неврология детского возраста 2017; 51(1):41–9.</mixed-citation></citation-alternatives></ref><ref id="B6"><label>6.</label><citation-alternatives><mixed-citation xml:lang="en">Shatokhin Yu.V., Aslanyan K.S. Snezhko I.V. et al. Experience in the treatment of rare hereditary coagulopathies. Glavniy vrach = Chief Doctor. Hematology 2018;62(3):16–9. Available from: https://cyberleninka.ru/article/n/opyt-lecheniya-redkihnasledstvennyh-koagulopatiy. (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Шатохин Ю.В., Асланян К.С., Снежко И.В. и др. Опыт лечения редких наследственных коагулопатий. Главный врач. Гематология 2018;62(3): 16–9. Доступно по: https://cyberleninka.ru/article/n/opyt-lecheniya-redkihnasledstvennyh-koagulopatiy.</mixed-citation></citation-alternatives></ref><ref id="B7"><label>7.</label><mixed-citation>Astermark J., Petrini P., Tengborn L. et al. Primary prophylaxis in severe haemophilia should bestarted at an early age but can be individualized. Brit J Haematology 1999;105(4);9–13.</mixed-citation></ref><ref id="B8"><label>8.</label><mixed-citation>Goun J.G., Byung C.L., Jong-Hee Ch. Pediatric stroke. J Kor Neurosurg Soc 2015;57(6):396–400. DOI:10.3340/jkns.2015.57.6.396.</mixed-citation></ref><ref id="B9"><label>9.</label><mixed-citation>Jae J.L., Soo H.Y.. Ki H.C. et al. Spontaneous spinal epidural hematoma in an infant: A case report and review of the literature. J Korean Neurosurg Soc 2008;44(2):84–7. DOI:10.3340/jkns.2008.44.2.84.</mixed-citation></ref><ref id="B10"><label>10.</label><mixed-citation>Ljung R.C.R. Intracranial haemorrhage in haemophilia A and B. Brit J Haematol 2008;140(4):378–84. DOI:10.1111/j.1365-2141.2007.06949.x.</mixed-citation></ref><ref id="B11"><label>11.</label><mixed-citation>Nilay N., Harish P., Jagdish Ch. Spinal epidural hematoma in a patient with hemophilia b presenting as acute abdomen. Indian J Hematol Blood Transfus 2014;30(1):54–6. DOI:10.1007/s12288-013-0245-4.</mixed-citation></ref><ref id="B12"><label>12.</label><mixed-citation>Podolsky-Gondim G.G., Furlanetti L.L., Viana D.C. et al. The role of coagulopathy on clinical outcome following traumatic brain injury in children: analysis of 66 consecutive cases in a single center institution. Childs Nerv Syst 2018;34(12):2455–61. DOI:10.1007/s00381-018-3989-1.</mixed-citation></ref><ref id="B13"><label>13.</label><mixed-citation>Darby S.C., Wan Kan S., Spooner R.J. et al. Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood 2007; 110(3):815–25. DOI:10.1182/blood-2006-10-050435.</mixed-citation></ref><ref id="B14"><label>14.</label><mixed-citation>Borkar S.A., Prasad G.L., Satyarthee G.D. et al. Spontaneous spinal extradural hematoma in a child with hemophilia B, surgery or medical management – A dilemma? J Pediatr Neurosci 2011;6(2)131–3. DOI:10.4103/1817-1745.92837.</mixed-citation></ref><ref id="B15"><label>15.</label><mixed-citation>Tran T.Q., Heaton H.A. Online CME Pediatric Emergency Medicine Reports. Pediatric Stroke 2015;20(10). Available at: https://www.reliasmedia.com/articles/136286.</mixed-citation></ref></ref-list></back></article>
