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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">urmj</journal-id><journal-title-group><journal-title xml:lang="ru">Уральский медицинский журнал</journal-title><trans-title-group xml:lang="en"><trans-title>Ural Medical Journal</trans-title></trans-title-group></journal-title-group><issn pub-type="epub">2949-4389</issn><publisher><publisher-name>Ural State Medical University</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.52420/umj.24.4.128</article-id><article-id custom-type="edn" pub-id-type="custom">USHHCB</article-id><article-id custom-type="elpub" pub-id-type="custom">urmj-1940</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>Оригинальные статьи | Original articles</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>Original articles</subject></subj-group></article-categories><title-group><article-title>Влияние CFTR-модуляторов на микробиом дыхательных путей и потребность в антибактериальной терапии у детей с муковисцидозом</article-title><trans-title-group xml:lang="en"><trans-title>The Impact of CFTR Modulators on the Airway Microbiome and the Need for Antibacterial Therapy in Children with Cystic Fibrosis</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0002-8869-108X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Васенёва</surname><given-names>Ю. О.</given-names></name><name name-style="western" xml:lang="en"><surname>Vasenyova</surname><given-names>Yu. O.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Васенёва Юлия Олеговна — ассистент кафедры госпитальной педиатрии, институт педиатрии и репродуктивной медицины, УГМУ; пульмонолог детского-консультативно-диагностического центра, Областная ДКБ.</p><p>Екатеринбург</p></bio><bio xml:lang="en"><p>Yulia O. Vaseneva — Assistant of the Department of Hospital Pediatrics, Institute of Pediatrics and Reproductive Medicine, Ural State Medical University; Pulmonologist of the Children’s Consultative Diagnostic Center, Regional Children’s Clinical Hospital.</p><p>Ekaterinburg</p></bio><email xlink:type="simple">yulya.vasenyova@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/0000-0002-5274-4699</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Вахлова</surname><given-names>И. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Vakhlova</surname><given-names>I. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Вахлова Ирина Вениаминовна — доктор медицинских наук, профессор, заведующий кафедрой госпитальной педиатрии, директор института педиатрии и репродуктивной медицины.</p><p>Екатеринбург</p></bio><bio xml:lang="en"><p>Irina V. Vakhlova — Doctor of Sciences (Medicine), Professor, Head of the Department of Hospital Pediatrics, Director of the Institute of Pediatrics and Reproductive Medicine, Ural State Medical University.</p><p>Ekaterinburg</p></bio><email xlink:type="simple">pediatria@usma.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/0000-0003-1630-1628</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ворошилина</surname><given-names>Е. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Voroshilina</surname><given-names>E. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Ворошилина Екатерина Сергеевна — доктор медицинских наук, профессор, заведующий кафедрой медицинской микробиологии и клинической лабораторной диагностики, институт профилактической медицины.</p><p>Екатеринбург</p></bio><bio xml:lang="en"><p>Ekaterina S. Voroshilina — Doctor of Sciences (Medicine), Professor, Head of the Department of Medical Microbiology and Clinical Laboratory Diagnostics, Institute of Preventive Medicine, Ural State Medical University.</p><p>Ekaterinburg</p></bio><email xlink:type="simple">voroshilina@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-9132-215X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Зорников</surname><given-names>Д. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Zornikov</surname><given-names>D. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Зорников Данила Леонидович — кандидат медицинских наук, доцент, доцент кафедры медицинской микробиологии и клинической лабораторной диагностики, институт профилактической медицины.</p><p>Екатеринбург</p></bio><bio xml:lang="en"><p>Danila L. Zornikov — Candidate of Sciences (Medicine), Associate Professor, Associate Professor of the Department of Medical Microbiology and Clinical Laboratory Diagnostics, Institute of Preventive Medicine, Ural State Medical University.</p><p>Ekaterinburg</p></bio><email xlink:type="simple">zornikovdl@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Уральский государственный медицинский университет; Областная детская клиническая больница</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Ural State Medical University; Regional Children’s Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Уральский государственный медицинский университет</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Ural State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2025</year></pub-date><pub-date pub-type="epub"><day>27</day><month>08</month><year>2025</year></pub-date><volume>24</volume><issue>4</issue><elocation-id>128–141</elocation-id><permissions><copyright-statement>Copyright &amp;#x00A9; Васенёва Ю.О., Вахлова И.В., Ворошилина Е.С., Зорников Д.Л., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Васенёва Ю.О., Вахлова И.В., Ворошилина Е.С., Зорников Д.Л.</copyright-holder><copyright-holder xml:lang="en">Vasenyova Y.O., Vakhlova I.V., Voroshilina E.S., Zornikov D.L.</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.umjusmu.ru/jour/article/view/1940">https://www.umjusmu.ru/jour/article/view/1940</self-uri><abstract><sec><title>Обоснование</title><p>Обоснование. Одним из основных критериев тяжести состояния пациентов с муковисцидозом является бактериальный состав бронхиального секрета, в связи с этим необходимы регулярный мониторинг микробного пейзажа и рациональное использование антибактериальной терапии (АБТ) у этой категории пациентов.</p><p>Цель — оценить влияние применения CFTR-модуляторов на микробиом дыхательных путей и потребность в АБТ у детей с муковисцидозом.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. Проведено открытое проспективное сравнительное нерандомизированное исследование в период с ноября 2023 г. по май 2024 г. В исследование включено 46 детей с муковисцидозом 2–17 лет, из которых 26 человек получали элексакафтор/тезакафтор/ивакафтор и 20 детей не принимали CFTR-модулятор. Оценка микробиологического состава дыхательных путей проводилась на старте и через 6 мес. наблюдения. Дополнительно изучались продолжительность и кратность парентеральной АБТ в обеих группах детей за 6 мес. до начала наблюдения и в течение 6 мес. после старта исследования.</p></sec><sec><title>Результаты</title><p>Результаты. Применение терапии CFTR-модуляторами способствовало снижению продолжительности курсов АБТ (р = 0,000), а отсутствие терапии, напротив, приводило к ее увеличению (р = 0,003). Существенных различий в частоте выявления отдельных групп бактерий в образцах орофарингеального мазка между группами, принимавшими и не принимавшими таргетную терапию, в динамике не выявлено. Обнаружена тенденция к увеличению количества бактериальной ДНК (р = 0,099) у пациентов, не принимавших CFTR-модулятор. Выявлено нарастание концентрации группы Enterobacteriales у пациентов без таргетной терапии в течение 6 мес. (р = 0,005).</p></sec><sec><title>Заключение</title><p>Заключение. На фоне терапии CFTR-модуляторами выявлено статистически значимое снижение потребности в парентеральной АБТ у детей с муковисцидозом. Несмотря на отсутствие достоверной разницы в частоте выявления большей части микроорганизмов, использование ПЦР-РВ позволило выявить колонизацию дыхательных путей у пациентов клинически значимыми бактериями: Staphylococcus aureus, Pseudomonas aeruginosa, Haemophilus influenzae.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Background</title><p>Background. One of the main criteria for the severity of the condition of patients with cystic fibrosis is the bacterial composition of bronchial secretions; in this regard, regular monitoring of the microbial landscape and rational use of antibacterial therapy in this category of patients are necessary.</p><p>The aim of the study is to assess the impact of CFTR modulators on the respiratory microbiome and the need for antibacterial therapy in children with cystic fibrosis.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. From November 2023 to May 2024, an open prospective study compared 46 children with cystic fibrosis (ages 2–17), including 26 on elexacaftor/tezacaftor/ivacaftor and 20 without CFTR modulators. Microbial composition and antibiotic therapy use were evaluated over 6-month pre- and post-study initiation.</p></sec><sec><title>Results</title><p>Results. The use of CFTR modulator contributed to a decrease in the duration of ABT courses (p = 0.000), while the absence of therapy, on the contrary, led to an increase in the duration of ABT courses (p = 0.003). No significant differences in the frequency of detection of individual bacterial groups between the groups were found over time. A tendency toward an increase in the amount of bacterial DNA (p = 0.099) was found in patients who didn’t take the CFTR modulator. An increase in the concentration of Enterobacteriales was found in patients without targeted therapy during 6 months of observation (p = 0.005).</p></sec><sec><title>Conclusion</title><p>Conclusion. CFTR modulator therapy significantly reduced ABT needs in cystic fibrosis children. Although no major differences in microbial detection frequencies were found, PCR identified clinically relevant bacterial colonizations: Staphylococcus aureus, Pseudomonas aeruginosa, Haemophilus influenzae.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>кистозный фиброз</kwd><kwd>дети</kwd><kwd>CFTR-модулятор</kwd><kwd>микробиом</kwd><kwd>ивакафтор</kwd><kwd>лумакафтор</kwd><kwd>тезакафтор</kwd><kwd>элексакафтор</kwd></kwd-group><kwd-group xml:lang="en"><kwd>cystic fibrosis</kwd><kwd>children</kwd><kwd>CFTR modulator</kwd><kwd>microbiome</kwd><kwd>ivacaftor</kwd><kwd>lumacaftor</kwd><kwd>tezacaftor</kwd><kwd>elecsacaftor</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">Palla J, Thapa S, Venkatachalam A, Runge JK, Laguna TA, Luna RA. The upper airway microbiome in Hispanic children with cystic fibrosis. Pediatric Pulmonology. 2023;58(8):2298–2307. 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