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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">rosped</journal-id><journal-title-group><journal-title xml:lang="ru">Российский педиатрический журнал имени М.Я. Студеникина</journal-title><trans-title-group xml:lang="en"><trans-title>M.Ya. Studenikin Russian Pediatric Journal</trans-title></trans-title-group></journal-title-group><publisher><publisher-name>ФГАУ «НМИЦ здоровья детей» Минздрава России</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.46563/1560-9561-2022-25-1-32-38</article-id><article-id custom-type="edn" pub-id-type="custom">hnsdoc</article-id><article-id custom-type="elpub" pub-id-type="custom">rosped-348</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>СОЦИАЛЬНАЯ ПЕДИАТРИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>SOCIAL PEDIATRICS</subject></subj-group></article-categories><title-group><article-title>Kачество жизни детей с муковисцидозом при нарушениях углеводного обмена</article-title><trans-title-group xml:lang="en"><trans-title>Quality of life of children with cystic fibrosis with disorders of carbohydrate metabolism</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4366-4467</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Лябина</surname><given-names>Надежда Вадимовна</given-names></name><name name-style="western" xml:lang="en"><surname>Lyabina</surname><given-names>Nadezhda V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-педиатр пульмонологического отделения, аспирант ФГАУ «НМИЦ здоровья детей» Минздрава Росси.</p><p>e-mail: marusya.1010@mail.ru</p></bio><email xlink:type="simple">marusya.1010@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/0000-0002-8750-9285</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Черников</surname><given-names>Владислав Владимирович</given-names></name><name name-style="western" xml:lang="en"><surname>Chernikov</surname><given-names>Vladislav V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Канд. мед. наук, ст. науч. сотр., зав. Центром методического аккредитационно-симуляционного центра Института подготовки медицинских кадров ФГАУ «НМИЦ здоровья детей» Минздрава России.</p><p>e-mail: vladfirst1@gmail.com</p></bio><email xlink:type="simple">vladfirst1@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2367-9920</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Симонова</surname><given-names>Ольга Игоревна</given-names></name><name name-style="western" xml:lang="en"><surname>Simonova</surname><given-names>Olga I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Доктор мед. наук, зав. пульмонологическим отд-нием ФГАУ «НМИЦ здоровья детей» Минздрава России.</p><p>e-mail: oisimonova@mail.ru</p></bio><bio xml:lang="en"><p>MD, PhD, DSci., Head of the Pulmonological department of the National Medical Research Center for Children’s Health, Moscow, 119991, Russian Federation.</p><p>e-mail: oisimonova@mail.ru</p></bio><email xlink:type="simple">oisimonova@mail.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/0000-0001-8743-0844</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Широкова</surname><given-names>Ирина Васильевна</given-names></name><name name-style="western" xml:lang="en"><surname>Shirokova</surname><given-names>Irina V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Канд. мед. наук, врач-эндокринолог консультативного отделения ФГАУ «НМИЦ здоровья детей» Минздрава России.</p><p>e-mail: irvash@list.ru</p></bio><email xlink:type="simple">irvash@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/0000-0002-4679-0533</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Смирнов</surname><given-names>Иван Евгеньевич</given-names></name><name name-style="western" xml:lang="en"><surname>Smirnov</surname><given-names>Ivan E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Доктор мед. наук, проф., начальник методического отдела «НМИЦ здоровья детей» Минздрава России.</p><p>e-mail: smirnov@nczd.ru</p></bio><email xlink:type="simple">smirnov@nczd.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/0000-0001-9838-3457</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Свиридова</surname><given-names>Татьяна Васильевна</given-names></name><name name-style="western" xml:lang="en"><surname>Sviridova</surname><given-names>Tatyana V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Канд. пед. наук, зав. лаб. специальной психологии и коррекционного обучения Центра психолого-педагогической помощи в педиатрии ФГАУ «НМИЦ здоровья детей» Минздрава России.</p><p>e-mail: tvsviridova@gmail.com</p></bio><email xlink:type="simple">tvsviridova@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГАУ «Национальный медицинский исследовательский центр здоровья детей» Минздрава России</institution></aff><aff xml:lang="en"><institution>National Medical Research Center for Children’s Health</institution></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФГАУ «Национальный медицинский исследовательский центр здоровья детей» Минздрава России; ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» (Сеченовский университет)</institution></aff><aff xml:lang="en"><institution>National Medical Research Center for Children’s Health; I.M. Sechenov Moscow State Medical University (Sechenov University)</institution></aff></aff-alternatives><pub-date pub-type="collection"><year>2022</year></pub-date><pub-date pub-type="epub"><day>11</day><month>10</month><year>2023</year></pub-date><volume>25</volume><issue>1</issue><fpage>32</fpage><lpage>38</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Лябина Н.В., Черников В.В., Симонова О.И., Широкова И.В., Смирнов И.Е., Свиридова Т.В., 2023</copyright-statement><copyright-year>2023</copyright-year><copyright-holder xml:lang="ru">Лябина Н.В., Черников В.В., Симонова О.И., Широкова И.В., Смирнов И.Е., Свиридова Т.В.</copyright-holder><copyright-holder xml:lang="en">Lyabina N.V., Chernikov V.V., Simonova O.I., Shirokova I.V., Smirnov I.E., Sviridova T.V.</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.rosped.ru/jour/article/view/348">https://www.rosped.ru/jour/article/view/348</self-uri><abstract><sec><title>Введение</title><p>Введение. Муковисцидоз (МВ) — наследственное, мультисистемное заболевание, которое сопровождается многочисленными осложнениями, что отражается на качестве жизни (КЖ) больных, определяя её продолжительность и комфортность. С увеличением выживаемости больных МВ выявлены различные сопутствующие заболевания, одним из которых являются МВ-ассоциированный (зависимый) сахарный диабет (МЗСД). Традиционно считается, что МЗСД ухудшает КЖ больного. Чтобы установить это, проводится анкетирование с валидированными опросниками для детей — Health Utilities Index (HUI). </p><p>Цель работы — определить КЖ больных МВ при нарушениях углеводного обмена.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. Проведено анкетирование 64 больных МВ в возрасте 5–18 лет (Ме = 13,59 года), из них 42 девочки и 22 мальчика. Для диагностики нарушений углеводного обмена всем больным проводился стандартный пероральный глюкозотолерантный тест, по данным которого больные МВ были распределены на 3 группы: 1-ю группу составили 25 больных МВ без нарушений углеводного обмена; 2-ю — 25 детей с предиабетом и 3-ю — 14 детей с МЗСД, которые находились на инсулинотерапии и без неё. Проведена оценка психологического состояния и развития больных МВ с предиабетом и МЗСД. Изучены параметры эмоционального благополучия, отношение к болезни и приверженность лечению. </p></sec><sec><title>Результаты</title><p>Результаты. Авторами не выявлено значимых различий КЖ больных МВ по всем шкалам опросника. Отмечена тенденция к снижению КЖ по общему баллу опросника (HUI3) у детей с МВ и предиабетом (0,77 ± 0,18 усл. ед.) по сравнению с больными без нарушений углеводного обмена и пациентами с МЗСД (0,81 ± 0,14 и 0,80 ± 0,14 усл. ед. соответственно). В эмоциональной сфере установлена тенденция к снижению КЖ больных МВ c предиабетом и МЗСД. Анализ индексов по отдельным шкалам выявил уменьшение КЖ по шкале «Эмоции» у больных МВ при нарушениях углеводного обмена. Больные МВ с МЗСД находятся в более тяжёлом психологическом состоянии, чем дети с предиабетом. Они значимо чаще испытывают психофизический дискомфорт и склонны к фиксации на симптомах болезни (критерий углового преобразования Фишера 3,11, p &lt; 0,01). Эти закономерности необходимо учитывать при анализе психоэмоционального состояния ребёнка с МВ для своевременной профилактики и коррекции.</p></sec><sec><title>Участие авторов</title><p>Участие авторов:Лябина Н.В., Симонова О.И., Свиридова Т.В. — концепция и дизайн исследования;Лябина Н.В. — сбор и обработка материала;Лябина Н.В., Черников В.В. — статистическая обработка;Лябина Н.В., Симонова О.И., Широкова И.В., Черников В.В. — написание текста;Смирнов И.Е., Свиридова Т.В. — редактирование.Все соавторы — утверждение окончательного варианта статьи, ответственность за целостность всех частей статьи. </p></sec><sec><title>Финансирование</title><p>Финансирование. Исследование не имело спонсорской поддержки.</p></sec><sec><title>Конфликт интересов</title><p>Конфликт интересов. Авторы заявляют об отсутствии конфликта интересов. </p></sec><sec><title>Поступила 14</title><p>Поступила 14.02.2022Принята к печати 17.02.2022Опубликована 15.03.2022</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. Cystic fibrosis (CF) is a hereditary, multisystem disease that is accompanied by numerous complications, which affects the quality of life (QOL) of patients, determining its duration and comfort. With an increase in the survival rate of CF patients, various concomitant diseases were identified, one of which is cystic fibrosis-associated (dependent) diabetes mellitus (CFDM). Traditionally, it is believed that CFDM worsens the patient’s QOL. To establish this, a questionnaire is conducted with validated questionnaires for children — Health Utilities Index (HUI).</p></sec><sec><title>Objective</title><p>Objective: to determine the QOL in CF patients with disorders of carbohydrate metabolism.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. There were surveyed sixty-four CF 5 to 18 year patients (Me = 13.59 years), including 42 girls and 22 boys. To diagnose disorders of carbohydrate metabolism, a standard oral glucose tolerance test (OGTT) was performed in all patients. According to OGTT data CF patients were divided into 3 groups: 1st — 25 cases without deteriorations of carbohydrate metabolism; 2nd — 25 children with prediabetes and 3rd — 14 CFDM patients on insulin therapy and without it. The assessment of the psychological state and development of CF patients with prediabetes and CFDM cases was carried out. The parameters of emotional well-being, attitude to the disease and adherence to treatment were studied.</p></sec><sec><title>Results</title><p>Results. The authors revealed no significant difference in the QOL in CF patients by all scales of the questionnaire. There was a tendency to decrease in QOL according to the overall score of the questionnaire (HUI3) in CF children with prediabetes (0.77 ± 0.18 units) compared with patients without carbohydrate metabolism disorders and CFDM patients (0.81 ± 0.14 units and 0.80 ± 0.14 units, respectively). In the emotional sphere, there is a tendency to decrease in QOL in patients with prediabetes and CFDM cases. The analysis of indices on separate scales revealed a more pronounced decrease in QOL on the emotion scale in CF patients with disorders of carbohydrate metabolism. CFDM patients are in a more severe psychological state than children with prediabetes. They are significantly more likely to experience psychophysical discomfort and tend to fixate on the symptoms of the disease (Fi­sher’s angular transformation criterion 3.11, p &lt; 0.01). These patterns should be taken into account when analyzing the psycho-emotional state of a CF child for timely prevention and correction.</p></sec><sec><title>Acknowledgement</title><p>Acknowledgement. The study had no sponsorship.</p></sec><sec><title>Conflict of interest</title><p>Conflict of interest. The authors declare no conflict of interest.</p></sec><sec><title>Received</title><p>Received: February 14, 2022Accepted: February 17, 2022Published: March 15, 2022</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>дети</kwd><kwd>муковисцидоз</kwd><kwd>качество жизни</kwd><kwd>психологический статус</kwd><kwd>нарушения углеводного обмена</kwd><kwd>предиабет</kwd><kwd>муковисцидоз-зависимый сахарный диабет</kwd></kwd-group><kwd-group xml:lang="en"><kwd>children</kwd><kwd>cystic fibrosis</kwd><kwd>quality of life</kwd><kwd>psychological status</kwd><kwd>carbohydrate metabolism disorders</kwd><kwd>prediabetes</kwd><kwd>cystic fibrosis-dependent diabetes mellitus</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">Ode K.L., Moran A. New insights into cystic fibrosis-related diabetes in children. 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