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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">vsp</journal-id><journal-title-group><journal-title xml:lang="ru">Вопросы современной педиатрии</journal-title><trans-title-group xml:lang="en"><trans-title>Current Pediatrics</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1682-5527</issn><issn pub-type="epub">1682-5535</issn><publisher><publisher-name>Издательство «ПедиатрЪ»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.15690/vsp.v20i6S.2372</article-id><article-id custom-type="elpub" pub-id-type="custom">vsp-2782</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>ОБМЕН ОПЫТОМ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>EXCHANGE OF EXPERIENCE</subject></subj-group></article-categories><title-group><article-title>Аллергические реакции при ферментозаместительной терапии детей с мукополисахаридозом, тип II</article-title><trans-title-group xml:lang="en"><trans-title>Allergic Reactions at Enzyme Replacement Therapy in Children with Mucopolysaccharidosis Type II</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2460-7718</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Левина</surname><given-names>Ю. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Levina</surname><given-names>Julia G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Левина Юлия Григорьевна, кандидат медицинских наук, заведующая отделом клинической иммунологии и аллергологии, врач аллерголог-иммунолог НИИ педиатрии и охраны здоровья детей ЦКБ РАН</p><p>119333, Москва, ул. Фотиевой, д. 10 стр. 1</p></bio><bio xml:lang="en"><p>Moscow</p></bio><email xlink:type="simple">julia.levina@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/0000-0001-8320-2027</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Вашакмадзе</surname><given-names>Н. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Vashakmadze</surname><given-names>Nato D.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><bio xml:lang="en"><p>Moscow</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2209-7531</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Намазова-Баранова</surname><given-names>Л. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Namazova-Baranova</surname><given-names>Leyla S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва; Белгород</p></bio><bio xml:lang="en"><p>Moscow; Belgorod</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7398-0562</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Вишнева</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Vishneva</surname><given-names>Elena A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><bio xml:lang="en"><p>Moscow</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6614-6115</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Журкова</surname><given-names>Н. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Zhurkova</surname><given-names>Natalia V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><bio xml:lang="en"><p>Moscow</p></bio><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0317-2425</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Эфендиева</surname><given-names>К. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Efendieva</surname><given-names>Kamilla E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><bio xml:lang="en"><p>Moscow</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5665-7835</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Алексеева</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Alekseeva</surname><given-names>Anna A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><bio xml:lang="en"><p>Moscow</p></bio><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3781-8661</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Калугина</surname><given-names>В. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Kalugina</surname><given-names>Vera G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва</p></bio><bio xml:lang="en"><p>Moscow</p></bio><xref ref-type="aff" rid="aff-4"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Научно-исследовательский институт педиатрии и охраны здоровья детей Центральной клинической больницы Российской академии наук; Российский национальный исследовательский медицинский университет им. Н.И. Пирогова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Pediatrics and Children’s Health in “Central Clinical Hospital of the Russian Academy of Sciences”; Pirogov Russian National Research Medical University</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 Центральной клинической больницы Российской академии наук; Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Белгородский государственный национальный исследовательский университет</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Pediatrics and Children’s Health in “Central Clinical Hospital of the Russian Academy of Sciences”; Pirogov Russian National Research Medical University; Belgorod State National Research University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Научно-исследовательский институт педиатрии и охраны здоровья детей Центральной клинической больницы Российской академии наук; Медико-генетический научный центр им. академика Н.П. Бочкова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Pediatrics and Children’s Health in “Central Clinical Hospital of the Russian Academy of Sciences”; Medical Genetic Research Center named after N.P. Bochkov</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>Научно-исследовательский институт педиатрии и охраны здоровья детей Центральной клинической больницы Российской академии наук</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Pediatrics and Children’s Health in “Central Clinical Hospital of the Russian Academy of Sciences”</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2021</year></pub-date><pub-date pub-type="epub"><day>17</day><month>12</month><year>2021</year></pub-date><volume>20</volume><issue>6s</issue><fpage>624</fpage><lpage>629</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Левина Ю.Г., Вашакмадзе Н.Д., Намазова-Баранова Л.С., Вишнева Е.А., Журкова Н.В., Эфендиева К.Е., Алексеева А.А., Калугина В.Г., 2021</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="ru">Левина Ю.Г., Вашакмадзе Н.Д., Намазова-Баранова Л.С., Вишнева Е.А., Журкова Н.В., Эфендиева К.Е., Алексеева А.А., Калугина В.Г.</copyright-holder><copyright-holder xml:lang="en">Levina J.G., Vashakmadze N.D., Namazova-Baranova L.S., Vishneva E.A., Zhurkova N.V., Efendieva K.E., Alekseeva A.A., Kalugina V.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://vsp.spr-journal.ru/jour/article/view/2782">https://vsp.spr-journal.ru/jour/article/view/2782</self-uri><abstract><p>Мукополисахаридоз, тип II (МПС II; синдром Хантера) — редкое наследственное заболевание, вызванное изменениями в структуре гена IDS и связанным с ними дефицитом лизосомного фермента идуронат-2-сульфатазы (I2S). Основой лечения детей с МПС II является ферментозаместительная терапия (ФЗТ) с рекомбинантной человеческой I2S. Одной из основных проблем ФЗТ является развитие аллергических, в том числе тяжелых анафилактических, реакций на рекомбинантные ферменты. В статье рассмотрены особенности инфузионных реакций гиперчувствительности при ФЗТ, описаны патогенез, диагностические критерии и алгоритм лечения анафилаксии. Обоснована необходимость дальнейших исследований аллергических инфузионных реакций у детей при ФЗТ.</p></abstract><trans-abstract xml:lang="en"><p>Mucopolysaccharidosis type II (MPS II; Hunter syndrome) is rare hereditary disease caused by changes in the IDS gene and associated deficiency of lysosomal enzyme iduronate-2-sulfatase (I2S). The main treatment scheme for children with MPS II is enzyme replacement therapy (ERT) with recombinant human I2S. The major issue of ERT is development of allergic (sometimes up to severe anaphylaxis) reactions to recombinant enzymes. The article covers features of infusion-related reactions to ERT, it describes pathogenesis, diagnostic criteria management algorithm of anaphylaxis. Whereas, there is the need of further studies on allergic infusion-related reactions to ERT in children.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>мукополисахаридоз</kwd><kwd>тип II</kwd><kwd>ферментозаместительная терапия</kwd><kwd>идурсульфаза</kwd><kwd>идурсульфаза бета</kwd><kwd>инфузионные реакции</kwd><kwd>анафилаксия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>mucopolysaccharidosis type II</kwd><kwd>enzyme replacement therapy</kwd><kwd>idursulfase</kwd><kwd>idursulfase beta</kwd><kwd>infusion-related reactions</kwd><kwd>anaphylaxis</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">Sestito S, Ceravolo F, Grisolia M, et al. Profile of idursulfase for the treatment of Hunter syndrome. 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