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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">ppharm</journal-id><journal-title-group><journal-title xml:lang="ru">Педиатрическая фармакология</journal-title><trans-title-group xml:lang="en"><trans-title>Pediatric pharmacology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1727-5776</issn><issn pub-type="epub">2500-3089</issn><publisher><publisher-name>Издательство «ПедиатрЪ»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.15690/pf.v12i2.1285</article-id><article-id custom-type="elpub" pub-id-type="custom">ppharm-11</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>В ПРАКТИКУ ПЕДИАТРА</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>For Pediatricians' Practice</subject></subj-group></article-categories><title-group><article-title>ОПЫТ ПРИМЕНЕНИЯ ИНГИБИТОРА ИНТЕРЛЕЙКИНА 1 КАНАКИНУМАБА У ПАЦИЕНТОВ С АУТОВОСПАЛИТЕЛЬНЫМИ ЗАБОЛЕВАНИЯМИ</article-title><trans-title-group xml:lang="en"><trans-title>CASE OF USING THE INHIBITOR OF INTERLEUKIN 1 CANACINUMAB IN PATIENTS WITH AUTO INFLAMMATORY DISEASES</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Салугина</surname><given-names>С. О.</given-names></name><name name-style="western" xml:lang="en"><surname>Salugina</surname><given-names>S. O.</given-names></name></name-alternatives><bio xml:lang="ru"><sec><title> </title></sec></bio><email xlink:type="simple">pafon1@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Фёдоров</surname><given-names>Е. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Fedorov</surname><given-names>E. S.</given-names></name></name-alternatives><email xlink:type="simple">pafon1@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кузьмина</surname><given-names>Н. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Kuz'mina</surname><given-names>N. N.</given-names></name></name-alternatives><email xlink:type="simple">pafon1@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Захарова</surname><given-names>Е. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Zakharova</surname><given-names>E. Yu.</given-names></name></name-alternatives><email xlink:type="simple">pafon1@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 ревматологии имени В.А. Насоновой, Москва, Российская Федерация</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Nasonova Rheumatology Scientific Research Center, Moscow, Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Медико-генетический научный центр, Москва, Российская Федерация</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Medico-Genetic Scientific Center, Moscow, Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2015</year></pub-date><pub-date pub-type="epub"><day>19</day><month>04</month><year>2015</year></pub-date><volume>12</volume><issue>2</issue><issue-title>Педиатрическая фармакология</issue-title><fpage>209</fpage><lpage>217</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Салугина С.О., Фёдоров Е.С., Кузьмина Н.Н., Захарова Е.Ю., 2015</copyright-statement><copyright-year>2015</copyright-year><copyright-holder xml:lang="ru">Салугина С.О., Фёдоров Е.С., Кузьмина Н.Н., Захарова Е.Ю.</copyright-holder><copyright-holder xml:lang="en">Salugina S.O., Fedorov E.S., Kuz'mina N.N., Zakharova E.Y.</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.pedpharma.ru/jour/article/view/11">https://www.pedpharma.ru/jour/article/view/11</self-uri><abstract><p>Основным медиатором воспаления для многих аутовоспалительных заболеваний (АВЗ) является интерлейкин 1β (IL 1β). Криопиринассоциированные периодические синдромы (CAPS) стали первыми заболеваниями, при которых с высокой степенью доказательности была показана эффективность ингибиторов IL 1. Канакинумаб (полностью человеческие моноклональные антитела к IL 1β) с 2011 г. зарегистрирован в Российской Федерации для лечения CAPS. В настоящее время проводятся исследования по оценке эффективности и переносимости ингибиторов IL 1 у пациентов с другими АВЗ. Накопленный практический опыт и немногочисленные рандомизированные контролируемые исследования демонстрируют успешное применение ингибиторов IL 1 у колхицинрезистентных пациентов с семейной средиземноморской лихорадкой с купированием воспалительных атак и снижением острофазовой активности, а также у больных с другими моногенными (TRAPS, HIDS и др.) и мультифакториальными патологиями (системный ювенильный артрит, болезнь Стилла у взрослых, подагра и др.). Применение ингибиторов IL 1, в частности канакинумаба, у пациентов с различными АВЗ, по данным российских и зарубежных исследований, показало хорошую переносимость и высокую эффективность препарата у всех больных независимо от возраста. Таким образом, канакинумаб, благодаря своим терапевтическим возможностям, имеет широкие перспективы в плане облегчения течения болезни, улучшения выживаемости, качества жизни и общего прогноза.</p></abstract><trans-abstract xml:lang="en"><p>Interleukin 1 (IL 1) is the main mediator for many auto inflammatory diseases (AID). Cryopyrin-associated periodic syndromes (CAPS) became the first diseases, for which the efficacy of IL 1 inhibitors was shown with a high degree of evidence. Canacinumb (totally human monoclonal antibodies to IL 1 is registered in Russia since 2011 for treating CAPS. At the moment research is being conducted to evaluate the efficacy and tolerance to IL 1 inhibitors in patients with other AIDs. The accumulated experience and scarce randomizedcontrolled studies demonstrate a successful usage of IL 1 inhibitors in colchicine-resistant patients with family Mediterranean fever with the cupping of inflammation attacks, reducing the acute phase activity and also in patients with other monogenic (TRAPS, HIDS and others) and multifactorial pathologies (systemic juvenile arthritis, Stilk disease in adults, gout etc.). Using the IL 1 inhibitors, especially canacinumab, in patients with different AIDs has shown good tolerance and a high efficacy in all patients with no correlation to the age, according to Russian and world research. Thus, canacinumab, thanks to its therapeutic abilities, has broad perspectives in terms of lightening the disease course, improving survival, life quality and the overall prognosis.</p><p> </p></trans-abstract><kwd-group xml:lang="ru"><kwd>криопиринассоциированные периодические синдромы</kwd><kwd>периодический синдром</kwd><kwd>ассоциированный с мутацией гена рецептора фактора некроза опухоли</kwd><kwd>лечение</kwd><kwd>канакинумаб</kwd><kwd>ингибиторы IL 1</kwd></kwd-group><kwd-group xml:lang="en"><kwd>cryopyrin-associated periodic syndromes</kwd><kwd>periodic syndrome</kwd><kwd>associated with the tumor necrosis factor receptor gene  mutation</kwd><kwd>treatment</kwd><kwd>canakinumab</kwd><kwd>IL 1inhibitors</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">Federici S., Gattorno M. A practical approach to the diagnosis of autoinflammatory diseases in childhood. 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