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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">pulmo</journal-id><journal-title-group><journal-title xml:lang="ru">Пульмонология</journal-title><trans-title-group xml:lang="en"><trans-title>PULMONOLOGIYA</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">0869-0189</issn><issn pub-type="epub">2541-9617</issn><publisher><publisher-name>Scientific and Practical Journal “PULMONOLOGIYA” LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.18093/0869-0189-2018-28-6-754-761</article-id><article-id custom-type="elpub" pub-id-type="custom">pulmo-1093</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>ОБЗОРЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEW</subject></subj-group></article-categories><title-group><article-title>Особенности заболеваний верхних дыхательных путей при муковисцидозе: методы лечения</article-title><trans-title-group xml:lang="en"><trans-title>Treatment of upper airway diseases in patients with cystic fibrosis</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>Шумкова</surname><given-names>Г. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Shumkova</surname><given-names>G. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Шумкова Галина Леонидовна – научный сотрудник Центра муковисцидоза НИИП; аспирант кафедры болезней уха, горла и носа, Сеченовский Университет.</p><p>119991, Москва, ул. Трубецкая, 8, стр. 2; 115682, Москва, Ореховый бульвар, 28, тел.: (499) 248-55-38</p></bio><bio xml:lang="en"><p>Galina L. Shumkova - Researcher, Center of Cystic Fibrosis, Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; Postgraduate Student at Department of Ear, Nose, and Throat Diseases Sechenov University, Healthcare MR</p><p>Trubetskaya ul. 8, build. 2, Moscow, 119991, Orekhovyy bul'var 28, Moscow, 115682,tel.: (499) 248-55-38</p></bio><email xlink:type="simple">galina2212@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>Амелина</surname><given-names>Е. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Amelina</surname><given-names>E. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Амелина Елена Львовна – кандидат медицинских наук, заведующая лабораторией муковисцидоза.</p><p>115682, Москва, Ореховый бульвар, 28, тел.: (926) 205-03-91</p></bio><bio xml:lang="en"><p>Elena L. Amelina - Candidate of Medicine, Head of Laboratory of Cystic Fibrosis.</p><p>Orekhovyy bul'var 28, Moscow, 115682, tel.: (926) 205-03-91</p><p> </p></bio><email xlink:type="simple">eamelina@mail.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>Свистушкин</surname><given-names>В. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Svistushkin</surname><given-names>V. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Свистушкин Валерий Михайлович – доктор медицинских наук, профессор, директор Клиники болезней уха, горла и носа, заведующий кафедрой болезней уха, горла и носа, Сеченовский Университет, Главный внештатный оториноларинголог Центрального федерального округа России.</p><p>119991, Москва, ул. Трубецкая, 8, стр. 2, тел.: (499) 248-55-11</p></bio><bio xml:lang="en"><p>Valeriy M. Svistushkin - Doctor of Medicine, Professor, Director of Clinics of Ear, Nose, and Throat Diseases, Head of Department of Ear, Nose, and Throat Diseases, Sechenov University; Chief Otolaryngologist at the Central Federal District of Russia.</p><p>Trubetskaya ul. 8, build. 2, Moscow, 119991, tel.: 499) 248-55-11</p></bio><email xlink:type="simple">svvm3@yandex.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Красовский</surname><given-names>С. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Krasovskiy</surname><given-names>S. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Красовский Станислав Александрович – кандидат медицинских наук, старший научный сотрудник лаборатории муковисцидоза.</p><p>115682, Москва, Ореховый бульвар, 28, тел.: (495) 965-23-24</p></bio><bio xml:lang="en"><p>Stanislav A. Krasovskiy - Candidate of Medicine, Senior Researcher, Laboratory of Cystic Fibrosis.</p><p>Orekhovyy bul'var 28, Moscow, 115682, tel.: (495) 965-23-24</p></bio><email xlink:type="simple">sa_krasovsky@mail.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>Каширская</surname><given-names>Н. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Kashirskaya</surname><given-names>N. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Каширская Наталия Юрьевна – доктор медицинских наук, профессор, главный научный сотрудник лаборатории генетической эпидемиологии.</p><p>115478, Москва, ул. Москворечье, 1, тел.: (910) 440-05-63</p></bio><bio xml:lang="en"><p>Nataliya Yu. Kashirskaya - Doctor of Medicine, Professor, Principal Researcher at Laboratory of Genetic Epidemiology.</p><p>Ul. Moskvorech'e 1, Moscow, 1115478, tel.: (910) 440-05-63</p></bio><email xlink:type="simple">kashirskayanj@mail.ru</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Синьков</surname><given-names>Э. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Sin’kov</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Синьков Эдуард Викторович – кандидат медицинских наук, доцент кафедры болезней уха, горла и носа.</p><p>119991, Москва, ул. Трубецкая, 8, стр. 2, тел.: (499) 248-55-38</p></bio><bio xml:lang="en"><p>Eduard V. Sin’kov - Candidate of Medicine, Associate Professor, Department of Ear, Nose, and Throat Diseases.</p><p>Trubetskaya ul. 8, build. 2, Moscow, 119991, tel.: (499) 248-55-38</p></bio><email xlink:type="simple">1178461@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное образовательное учреждение высшего образования «Первый Московский государственный медицинский университет имени И.М.Сеченова» Министерства здравоохранения Российской Федерации (Сеченовский Университет); Федеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.M.Sechenov First Moscow State Medical University (Sechenov University), Healthcare Ministry of Russia; Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное образовательное учреждение высшего образования «Первый Московский государственный медицинский университет имени И.М.Сеченова» Министерства здравоохранения Российской Федерации (Сеченовский Университет)</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.M.Sechenov First Moscow State Medical University (Sechenov University), Healthcare Ministry of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное научное учреждение Медико-генетический научный центр</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Federal Medical Genetic Academic Center, Russian Academy of Science</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2018</year></pub-date><pub-date pub-type="epub"><day>16</day><month>02</month><year>2019</year></pub-date><volume>28</volume><issue>6</issue><fpage>754</fpage><lpage>761</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Шумкова Г.Л., Амелина Е.Л., Свистушкин В.М., Красовский С.А., Каширская Н.Ю., Синьков Э.В., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Шумкова Г.Л., Амелина Е.Л., Свистушкин В.М., Красовский С.А., Каширская Н.Ю., Синьков Э.В.</copyright-holder><copyright-holder xml:lang="en">Shumkova G.L., Amelina E.L., Svistushkin V.M., Krasovskiy S.A., Kashirskaya N.Y., Sin’kov E.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://journal.pulmonology.ru/pulm/article/view/1093">https://journal.pulmonology.ru/pulm/article/view/1093</self-uri><abstract><p>Основной патологией верхних дыхательных путей (ВДП) при муковисцидозе (МВ) является полипозно-гнойный риносинусит. Эта патология развивается в детстве и является следствием микробной контаминации застойной слизи околоносовых пазух с дальнейшим развитием инфекционно-воспалительного процесса и формированием назальных полипов. Наличие хронического очага инфекции в ВДП отрицательно сказывается на течении легочной патологии. Наблюдение оториноларингологом больных данной категории должно проводиться пожизненно, обследование должно включать в себя объективные методы обследования – компьютерную томографию пазух носа, эндоскопию, микробиологическое исследование содержимого назальных синусов. Для повышения эффективности лечения риносинусита при МВ следует объединять топическую ингаляционную терапию и эндоскопические малоинвазивные хирургические методы.</p></abstract><trans-abstract xml:lang="en"><p>Chronic rhinosinusitis with nasal polyps (CRS NP) is the most prevalent ear, nose, and throat (ENT) disorder in cystic fibrosis (CF) patients. CRS NP can occur in CF children at the age of ≥ 8 months and becomes the chronic disease. Tenacious secret and bacterial infection cause obstruction, edema and hypoxia of paranasal sinuses and contribute to significant morbidity in CF patients. The active monitoring and treatment of CRS NP aimed at improvement of the aeration of paranasal sinuses, mucociliary clearance, and the control of chronic upper airway infection are necessary. Functional Endoscopic Sinus Surgery (FESS) may be the method of choice if surgical treatment is considered. FESS facilitates paranasal sinuses drainage. Due to recurrent course of CRS NP in most CF patients, lifelong follow-up by an ENT specialist and postoperative management using topical steroids and/or antibiotics are required.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>муковисцидоз</kwd><kwd>полипозный риносинусит</kwd><kwd>патогенез</kwd><kwd>ингаляционные антибактериальные препараты</kwd><kwd>дорназа альфа</kwd><kwd>полипотомия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>purulent rhinosinusitis</kwd><kwd>cystic fibrosis</kwd><kwd>nasal polyps</kwd><kwd>inhalational therapy</kwd><kwd>sinusotomy</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">Oomen K., April M. Sinonasal manifestations in cystic fibrosis. Int. J. Otolaryngol. 2012; 2012: 789572. DOI: 10.1155/2012/789572.</mixed-citation><mixed-citation xml:lang="en">Oomen K., April M. Sinonasal manifestations in cystic fibrosis. Int. J. 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