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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.v12i4.1428</article-id><article-id custom-type="elpub" pub-id-type="custom">ppharm-516</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>ДИСКУССИОННЫЕ ВОПРОСЫ ФАРМАКОТЕРАПИИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>DEBATABLE ISSUES OF PHARMACOTHERAPY</subject></subj-group></article-categories><title-group><article-title>Высокая эффективность канакинумаба у пациента с поздно диагностированным криопиринассоциированным синдромом (синдром CINCA)</article-title><trans-title-group xml:lang="en"><trans-title>High Efficiency of Kanakinumabum for a Patient with a Late Diagnosed Chronic Infantile Neurological Cutaneous Articular Syndrome (CINCA)</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>Слепцова</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Sleptsova</surname><given-names>T. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Алексеева</surname><given-names>Е. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Alekseeva</surname><given-names>E. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>доктор медицинских наук, профессор, заведующая ревматологическим отделением ФГБНУ «НЦЗД» Минздрава России, декан педиатрического факультета Первого МГМУ им. И.М. Сеченова Адрес: 119991, Москва, Ломоносовский проспект, д. 2, стр. 1, тел.: +7 (499) 134-02-97</p></bio><email xlink:type="simple">alekatya@yandex.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Савостьянов</surname><given-names>К. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Savostianov</surname><given-names>K. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Пушков</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Pushkov</surname><given-names>A. A.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Бзарова</surname><given-names>Т. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Bazarova</surname><given-names>T. M.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Валиева</surname><given-names>С. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Valieva</surname><given-names>S. I.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ломакина</surname><given-names>О. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Lomakina</surname><given-names>O. L.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Денисова</surname><given-names>Р. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Denisova</surname><given-names>R. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Исаева</surname><given-names>К. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Isaeva</surname><given-names>K. B.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чистякова</surname><given-names>Е. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Chistyakova</surname><given-names>E. G.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Научный центр здоровья детей, Москва, Российская Федерация</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Scientific Center of Children’s Health, Moscow, Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Научный центр здоровья детей, Москва, Российская Федерация&#13;
&#13;
Первый Московский государственный медицинский университет им. И.М. Сеченова, Москва, Российская Федерация</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Scientific Center of Children’s Health, Moscow, Russian Federation&#13;
&#13;
I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Первый Московский государственный медицинский университет им. И.М. Сеченова, Москва, Российская Федерация</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.M. Sechenov First Moscow State Medical University, 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>03</day><month>11</month><year>2015</year></pub-date><volume>12</volume><issue>4</issue><fpage>456</fpage><lpage>561</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Слепцова Т.В., Алексеева Е.И., Савостьянов К.В., Пушков А.А., Бзарова Т.М., Валиева С.И., Ломакина О.Л., Денисова Р.В., Исаева К.Б., Чистякова Е.Г., 2015</copyright-statement><copyright-year>2015</copyright-year><copyright-holder xml:lang="ru">Слепцова Т.В., Алексеева Е.И., Савостьянов К.В., Пушков А.А., Бзарова Т.М., Валиева С.И., Ломакина О.Л., Денисова Р.В., Исаева К.Б., Чистякова Е.Г.</copyright-holder><copyright-holder xml:lang="en">Sleptsova T.V., Alekseeva E.I., Savostianov K.V., Pushkov A.A., Bazarova T.M., Valieva S.I., Lomakina O.L., Denisova R.V., Isaeva K.B., Chistyakova E.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://www.pedpharma.ru/jour/article/view/516">https://www.pedpharma.ru/jour/article/view/516</self-uri><abstract><p>статье представлено наблюдение диагностированного в поздние сроки тяжелого течения криопиринассоциированного синдрома (синдром CINCA/NOMID). Описано успешное применение моноклональных антител к IL 1 — канакинумаба — у пациента с хроническим неврологическим кожным и суставным синдромом: уже через 1 нед терапии полностью купировались лихорадка, кожный и болевой синдромы, уменьшились контрактуры в суставах. Через 8 нед лечения у ребенка нормализовались лабораторные показатели активности болезни (СОЭ и СРБ). Через 24 нед полностью восстановились движения в пораженных суставах; сурдологом отмечено улучшение слуха. Приведенный клинический пример демонстрирует высокую эффективность канакинумаба у больного хроническим неврологическим кожным и суставным синдромом и свидетельствует о перспективности терапевтического применения блокатора IL 1 у пациентов с криопиринассоциированным синдромом, в том числе на поздних стадиях заболевания. Нежелательных явлений на фоне терапии канакинумабом не отмечалось.</p></abstract><trans-abstract xml:lang="en"><p>The article presents the monitoring of a severe course of CINCA/NOMID syndrome diagnosed at late stages. The use of monoclonal antibodies to IL 1 — kanakinumabum — in a patient with chronic neurological cutaneous and articular syndrome was successful: fever, cutaneous and pain syndromes were completely stopped, joint contractures decreased after one week of therapy. Laboratory parameters of the child’s disease activity (ESR and CRP) became normal after 8 weeks of treatment. Movements in the affected joints recovered completely after 24 weeks; the audiologist noted an improvement in hearing. The above clinical example demonstrates the high efficiency of kanakinumabum for a patient with chronic neurological cutaneous and articular syndrome, and shows the perspective of therapeutic application of IL 1 blocker for patients with CINCA syndrome including advanced stages of the disease. No adverse effects were noted during kanakinumabum therapy.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>дети</kwd><kwd>аутовоспалительные синдромы</kwd><kwd>CINCA</kwd><kwd>криопиринассоциированный синдром</kwd><kwd>канакинумаб</kwd><kwd>человеческое моноклональное антитело к интерлейкину 1</kwd></kwd-group><kwd-group xml:lang="en"><kwd>children</kwd><kwd>autoinflammatory syndromes</kwd><kwd>CINCA</kwd><kwd>chronic infantile neurological cutaneous articular syndrome</kwd><kwd>kanakinumabun</kwd><kwd>human monoclonal antibody to 1 interleukin</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">Гатторно М. Аутовоспалительные заболевания у детей. Вопросы современной педиатрии. 2014; 13 (2): 55–64.</mixed-citation><mixed-citation xml:lang="en">Гатторно М. Аутовоспалительные заболевания у детей. 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