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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">medsovet</journal-id><journal-title-group><journal-title xml:lang="ru">Медицинский Совет</journal-title><trans-title-group xml:lang="en"><trans-title>Meditsinskiy sovet = Medical Council</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2079-701X</issn><issn pub-type="epub">2658-5790</issn><publisher><publisher-name>REMEDIUM GROUP Ltd.</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21518/ms2026-045</article-id><article-id custom-type="elpub" pub-id-type="custom">medsovet-9904</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>ТРУДНЫЙ ДИАГНОЗ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>DIFFICULT DIAGNOSIS</subject></subj-group></article-categories><title-group><article-title>Изменения в компонентном составе тела детей с муковисцидозом на фоне таргетной терапии: пилотное исследование</article-title><trans-title-group xml:lang="en"><trans-title>Body composition changes in children with cystic fibrosis while receiving targeted therapy: A pilot study</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9532-9698</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Завьялова</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Zavyalova</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Завьялова Анна Никитична - д.м.н., профессор кафедры пропедевтики детских болезней с курсом общего ухода за детьми, врач-диетолог Клиники.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Anna N. Zavyalova - Dr. Sci. (Med.), Professor of the Department of Propaedeutics of Childhood Diseases with a Course in General Child Care, Nutritionist at the Clinic, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</p></bio><email xlink:type="simple">anzavjalova@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/0000-0001-8986-7599</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Яковлева</surname><given-names>М. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Yakovleva</surname><given-names>M. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Яковлева Милена Николаевна - врач-диетолог Клиники.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Milena N Yakovleva - Nutritionist at the Clinic, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</p></bio><email xlink:type="simple">milena-yakovleva@bk.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/0000-0002-9029-2312</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Панкратова</surname><given-names>П. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Pankratova</surname><given-names>P. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Панкратова Полина Андреевна - ассистент кафедры пропедевтики детских болезней с курсом общего ухода за детьми.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Polina А. Pankratova - Assistant Professor of the Department of Propaedeutics of Childhood Diseases with a Course in General Child Care, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</p></bio><email xlink:type="simple">polina.pankratova@bk.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>Гоголев</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Gogolev</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Гоголев Андрей Викторович - ассистент кафедры пропедевтики детских болезней с курсом общего ухода за детьми.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Andrey V. Gogolev - Assistant Professor of the Department of Propaedeutics of Childhood Diseases with a Course in General Child Care, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</p></bio><email xlink:type="simple">gogadoc@list.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/0009-0002-5976-1448</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кобякова</surname><given-names>В. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Kobyakova</surname><given-names>V. D.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кобякова Виталина Денисовна - студент педиатрического факультета.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Vitalina D. Kobyakova - Student of the Pediatric Faculty, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</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/0009-0006-0379-1284</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Доренских</surname><given-names>П. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Dorenskikh</surname><given-names>P. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Доренских Полина Юрьевна - студент педиатрического факультета.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Polina Yu. Dorenskikh - Student of the Pediatric Faculty, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</p></bio><email xlink:type="simple">dorenskikh@bk.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/0009-0006-2779-3479</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Соломатина</surname><given-names>Н. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Solomatina</surname><given-names>N. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Соломатина Наталья Михайловна - врач-пульмонолог Клиники.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Natalia M. Solomatina - Pulmonologist at the Clinic, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</p></bio><email xlink:type="simple">nm.solomatina@gpmu.org</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>Пешехонова</surname><given-names>Ю. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Peshekhonova</surname><given-names>Yu. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Пешехонова Юлия Владимировна - к.м.н., заведующая отделением педиатрии №2 Клиники.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Yuliya V. Peshekhonova - Cand. Sci. (Med.), Head of the Department of Pediatrics No. 2 at the Clinic, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</p></bio><email xlink:type="simple">peshekhonova@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/0000-0002-0992-1709</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Новикова</surname><given-names>В. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Novikova</surname><given-names>V. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Новикова Валерия Павловна - д.м.н., профессор, заведующая кафедрой пропедевтики детских болезней с курсом общего ухода за детьми, заведующая лабораторией медико-социальных проблем в педиатрии Научно-исследовательского центра.</p><p>194100, Санкт-Петербург, ул. Литовская, д. 2</p></bio><bio xml:lang="en"><p>Valeria P. Novikova - Dr. Sci. (Med.), Professor, Head of the Department of Propaedeutics of Childhood Diseases with a Course in General Child Care, Head of the Laboratory of Medical and Social Problems in Pediatrics at the Research Center, St Petersburg State Pediatric Medical University.</p><p>2, Litovskaya St., St Petersburg, 194100</p></bio><email xlink:type="simple">novikova-vp@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru">Санкт-Петербургский государственный педиатрический медицинский университет<country>Россия</country></aff><aff xml:lang="en">St Petersburg State Pediatric Medical University<country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2026</year></pub-date><pub-date pub-type="epub"><day>24</day><month>03</month><year>2026</year></pub-date><volume>0</volume><issue>1</issue><fpage>217</fpage><lpage>224</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Завьялова А.Н., Яковлева М.Н., Панкратова П.А., Гоголев А.В., Кобякова В.Д., Доренских П.Ю., Соломатина Н.М., Пешехонова Ю.В., Новикова В.П., 2026</copyright-statement><copyright-year>2026</copyright-year><copyright-holder xml:lang="ru">Завьялова А.Н., Яковлева М.Н., Панкратова П.А., Гоголев А.В., Кобякова В.Д., Доренских П.Ю., Соломатина Н.М., Пешехонова Ю.В., Новикова В.П.</copyright-holder><copyright-holder xml:lang="en">Zavyalova A.N., Yakovleva M.N., Pankratova P.A., Gogolev A.V., Kobyakova V.D., Dorenskikh P.Y., Solomatina N.M., Peshekhonova Y.V., Novikova V.P.</copyright-holder><license 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.med-sovet.pro/jour/article/view/9904">https://www.med-sovet.pro/jour/article/view/9904</self-uri><abstract><sec><title>Введение</title><p>Введение. Основная терапия муковисцидоза носит консервативный характер и в первую очередь включает муколитическую и антибактериальную терапию. Большая роль отводится диетотерапии, которая направлена на коррекцию нутритивных нарушений. Она предусматривает заместительную терапию ферментами поджелудочной железы и применение высококалорийных энтеральных добавок, способствующих улучшению нутритивного статуса пациентов.</p></sec><sec><title>Цель</title><p>Цель. Изучить влияние таргетной терапии на изменения в компонентном составе тела детей с муковисцидозом.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. В одноцентровом ретроспективном исследовании были изучены 67 протоколов биоимпедансометрии (Диамант-АИСТ-мини) у 31 пациента в возрасте от 4 до 17 лет: 15 мальчиков (48%) и 16 девочек (52%). В рамках наблюдательного исследования пациенты были разделены на 2 группы: основную – 11 человек (35%), получавших таргетную терапию в течение 1 года, и группу сравнения – 20 детей (65%), у которых оценка нутритивного статуса проводилась до назначения таргетной терапии. Все дети получали заместительную ферментную терапию и нутритивную поддержку смесями для энтерального питания, рекомендованными для пациентов с муковисцидозом. Оценка компонентного состава тела проводилась ежегодно во время плановой госпитализации.</p></sec><sec><title>Результаты</title><p>Результаты. Значимых различий показателей Z-score роста, массы тела и индекса массы тела в группах не выявлено. Компонентный состав тела имел различия. При анализе жировой массы, ее доли и отклонений в доле сухой клеточной массы в зависимости от применения (неприменения) таргетной терапии были выявлены статистически значимые различия (p = 0,046; p = 0,020; p = 0,021 соответственно). На фоне таргетной терапии у пациентов отмечалось увеличение жировой массы в компонентном составе тела и ее доли на фоне роста доли сухой клеточной массы. В группе сравнения содержание жировой массы, ее доли и сухой клеточной массы было ниже возрастных нормативов.</p></sec><sec><title>Выводы</title><p>Выводы. Таргетная терапия улучшила контроль над заболеванием, но изменила компонентный состав тела у пациентов, ее получавших. Нутритивная поддержка остается необходимым методом лечения пациентов с муковисцидозом.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. The primary treatment for cystic fibrosis is conservative, primarily mucolytic and antibacterial. Dietary therapy plays a significant role, targeting mechanisms such as pancreatic enzyme replacement therapy and enteral caloric supplements that improve nutritional status.</p></sec><sec><title>Aim</title><p>Aim. To study the effect of targeted therapy on changes in the body composition of children with cystic fibrosis.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. A single-center retrospective study examined 67 bioimpedancemetry protocols (Diamant AIST mini) in 31 patients aged 4 to 17 years with cystic fibrosis receiving nutritional support before and after targeted therapy. There were 15 boys (48%) and 16 girls (52%). As part of the observational study, the patients were divided into 2 groups: 11 (35%) received targeted therapy for 1 year, and 20 children (65%) had their nutritional status assessed before targeted therapy. All children received enzyme replacement therapy and nutritional support with enteral nutrition mixtures recommended for patients with cystic fibrosis. Body composition assessments were performed annually during planned hospitalization.</p></sec><sec><title>Results</title><p>Results. No differences in the Z-score indicators of height, body weight and body mass index were found in the groups. The body composition varied. When using targeted therapy, statistically significant changes in fat mass, its proportion, and deviations in the proportion of dry cell mass were revealed (p = 0.046, p = 0.020, p = 0.021, respectively). With targeted therapy, patients demonstrated an increase in body fat mass and its proportion, along with an increase in the proportion of skeletal cell mass. In Group 2 without targeted therapy, fat mass, its proportion, and skeletal cell mass were below age-appropriate norms, despite adequate replacement therapy and nutritional support.</p></sec><sec><title>Conclusions</title><p>Conclusions. Targeted therapy improved disease control but altered body composition in patients receiving it. Nutritional support remains an essential treatment for patients with cystic fibrosis.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>биоимпедансометрия</kwd><kwd>дети</kwd><kwd>муковисцидоз</kwd><kwd>таргетная терапия</kwd><kwd>нутритивная поддержка</kwd></kwd-group><kwd-group xml:lang="en"><kwd>bioimpedancemetry</kwd><kwd>children</kwd><kwd>cystic fibrosis</kwd><kwd>targeted therapy</kwd><kwd>nutritional support</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">Баранов АА, Намазова-Баранова ЛС, Куцев СИ, Авдеев СН, Полевиченко ЕВ, Белевский АС и др. Кистозный фиброз (муковисцидоз): клинические рекомендации. М.; 2021. Режим доступа: https://library.mededtech.ru/rest/documents/KP372/.</mixed-citation><mixed-citation xml:lang="en">Баранов АА, Намазова-Баранова ЛС, Куцев СИ, Авдеев СН, Полевиченко ЕВ, Белевский АС и др. 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