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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">vsp</journal-id><journal-title-group><journal-title xml:lang="ru">Вопросы современной педиатрии</journal-title><trans-title-group xml:lang="en"><trans-title>Current Pediatrics</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1682-5527</issn><issn pub-type="epub">1682-5535</issn><publisher><publisher-name>Издательство «ПедиатрЪ»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.15690/vsp.v13i6.1209</article-id><article-id custom-type="elpub" pub-id-type="custom">vsp-109</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>ИНФОРМАЦИОННОЕ СООБЩЕНИЕ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>NEWS ITEM</subject></subj-group></article-categories><title-group><article-title>ПРОЕКТ НАЦИОНАЛЬНОГО КОНСЕНСУСА* «МУКОВИСЦИДОЗ: ОПРЕДЕЛЕНИЕ, ДИАГНОСТИЧЕСКИЕ КРИТЕРИИ, ТЕРАПИЯ». РАЗДЕЛ «ИНГАЛЯЦИОННАЯ ТЕРАПИЯ» (ПЕЧАТАЕТСЯ С СОКРАЩЕНИЯМИ)</article-title><trans-title-group xml:lang="en"><trans-title>THE NATIONAL CONSENSUS PROJECT* «CYSTIC FIBROSIS: DEFINITION, DIAGNOSTIC CRITERIA, AND THERAPY». SECTION «INHALATION THERAPY» (ABRIDGED)</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>Амелина</surname><given-names>Е. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Amelina</surname><given-names>Ye. L.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@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>Ашерова</surname><given-names>И. К.</given-names></name><name name-style="western" xml:lang="en"><surname>Asherova</surname><given-names>I. K.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@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>Волков</surname><given-names>И. К.</given-names></name><name name-style="western" xml:lang="en"><surname>Volkov</surname><given-names>I. K.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@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>Гембицкая</surname><given-names>Т. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Gembitskaya</surname><given-names>Т. Ye.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.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>Гинтер</surname><given-names>Е. К.</given-names></name><name name-style="western" xml:lang="en"><surname>Ginter</surname><given-names>Ye. K.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ильенкова</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Il’yenkova</surname><given-names>N. А.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-6"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Капранов</surname><given-names>Н. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Kapranov</surname><given-names>N. I.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Каримова</surname><given-names>И. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Karimova</surname><given-names>I. P.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-7"/></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 МГНЦ</p></bio><email xlink:type="simple">kashirskayanj@mail.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кондратьева</surname><given-names>Е. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Kondrat’yeva</surname><given-names>Ye. I.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Костылева</surname><given-names>М. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Kostyleva</surname><given-names>М. N.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-8"/></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>Krasovsky</surname><given-names>S. А.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@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>Мерзлова</surname><given-names>Н. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Merzlova</surname><given-names>N. B.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-9"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Назаренко</surname><given-names>Л. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Nazarenko</surname><given-names>L. P.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-10"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Намазова-Баранова</surname><given-names>Л. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Namazova-Baranova</surname><given-names>L. S.</given-names></name></name-alternatives><email xlink:type="simple">namazova@nczd.ru</email><xref ref-type="aff" rid="aff-11"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Неретина</surname><given-names>А. Ф.</given-names></name><name name-style="western" xml:lang="en"><surname>Neretina</surname><given-names>А. F.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-12"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Никонова</surname><given-names>В. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Nikonova</surname><given-names>V. S.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Орлов</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Orlov</surname><given-names>А. V.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-13"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Постников</surname><given-names>С. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Postnikov</surname><given-names>S. S.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-8"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Протасова</surname><given-names>Т. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Protasova</surname><given-names>Т. А.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-14"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Семыкин</surname><given-names>С. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Semykin</surname><given-names>S. Yu.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сергиенко</surname><given-names>Д. Ф.</given-names></name><name name-style="western" xml:lang="en"><surname>Sergienko</surname><given-names>D. F.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-15"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Симонова</surname><given-names>О. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Simonova</surname><given-names>О. I.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-16"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Успенская</surname><given-names>И. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Uspenskaya</surname><given-names>I. D.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-17"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чернуха</surname><given-names>М. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Chernukha</surname><given-names>М. Yu.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-18"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шабалова</surname><given-names>Л. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Shabalova</surname><given-names>L. А.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шагинян</surname><given-names>И. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Shaginyan</surname><given-names>I. А.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-18"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шерман</surname><given-names>В. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Sherman</surname><given-names>V. D.</given-names></name></name-alternatives><email xlink:type="simple">neonatepearl@yandex.ru</email><xref ref-type="aff" rid="aff-5"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Научно-исследовательский институт пульмонологии, Москва</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Pulmonology Research Institute, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Детская клиническая больница № 1, Ярославль</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Children's Clinical Hospital No.1, Yaroslavl</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,</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Sechenov First Moscow State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>НИИ пульмонологии СПбГМУ им. акад. И.П. Павлова, Санкт-Петербург</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Pulmonology Research Institute of Pavlov First Saint Petersburg State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>Медико-генетический научный центр, Москва</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Centre of Medical Genetics, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-6"><aff xml:lang="ru"><institution>Красноярский государственный медицинский университет им. проф. В.Ф. Войно-Ясенецкого</institution><country>Россия</country></aff><aff xml:lang="en"><institution>V.F. Voino-Yasenetsky Krasnoyarsk State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-7"><aff xml:lang="ru"><institution>Челябинская областная детская клиническая больница</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Chelyabinsk Regional Children's Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-8"><aff xml:lang="ru"><institution>Российская детская клиническая больница, Москва</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Russian Children's Clinical Hospital, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-9"><aff xml:lang="ru"><institution>Пермская государственная медицинская академия им. Е.А. Вагнера</institution><country>Россия</country></aff><aff xml:lang="en"><institution>E.A. Vagner Perm State Academy of Medicine</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-10"><aff xml:lang="ru"><institution>НИИ медицинской генетики, Томск</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute for Medical Genetics, Tomsk</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-11"><aff xml:lang="ru"><institution>Первый Московский государственный медицинский университет им. И.М. Сеченова;&#13;
Научный центр здоровья детей, Москва</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Scientific Centre of Children Health, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-12"><aff xml:lang="ru"><institution>Воронежская государственная медицинская академия им. Н.Н. Бурденко</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Burdenko Voronezh State Medical Academy</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-13"><aff xml:lang="ru"><institution>Детская городская больница Святой Ольги, Санкт-Петербург</institution><country>Россия</country></aff><aff xml:lang="en"><institution>St. Olga Municipal Children's Hospital, Saint Petersburg</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-14"><aff xml:lang="ru"><institution>Кемеровская областная клиническая больница</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Kemerovo Regional Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-15"><aff xml:lang="ru"><institution>Астраханская государственная медицинская академия</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Astrakhan State Medical Academy</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-16"><aff xml:lang="ru"><institution>Первый Московский государственный медицинский университет им. И.М. Сеченова;&#13;
Научный центр здоровья детей, Москва</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Sechenov First Moscow State Medical University;&#13;
Sechenov First Moscow State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-17"><aff xml:lang="ru"><institution>Нижегородский НИИ детской гастроэнтерологии</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Nizhny Novgorod Scientific Research Institute of Paediatric Gastroenterology</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-18"><aff xml:lang="ru"><institution>НИИ эпидемиологии и микробиологии им. Н.Ф. Гамалеи, Москва</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.F. Gamaleya Scientific Research Institute of Epidemiology and Microbiology, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2014</year></pub-date><pub-date pub-type="epub"><day>10</day><month>12</month><year>2014</year></pub-date><volume>13</volume><issue>6</issue><issue-title>Вопросы современной педиатрии</issue-title><fpage>89</fpage><lpage>95</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Амелина Е.Л., Ашерова И.К., Волков И.К., Гембицкая Т.Е., Гинтер Е.К., Ильенкова Н.А., Капранов Н.И., Каримова И.П., Каширская Н.Ю., Кондратьева Е.И., Костылева М.Н., Красовский С.А., Мерзлова Н.Б., Назаренко Л.П., Намазова-Баранова Л.С., Неретина А.Ф., Никонова В.С., Орлов А.В., Постников С.С., Протасова Т.А., Семыкин С.Ю., Сергиенко Д.Ф., Симонова О.И., Успенская И.Д., Чернуха М.Ю., Шабалова Л.А., Шагинян И.А., Шерман В.Д., 2014</copyright-statement><copyright-year>2014</copyright-year><copyright-holder xml:lang="ru">Амелина Е.Л., Ашерова И.К., Волков И.К., Гембицкая Т.Е., Гинтер Е.К., Ильенкова Н.А., Капранов Н.И., Каримова И.П., Каширская Н.Ю., Кондратьева Е.И., Костылева М.Н., Красовский С.А., Мерзлова Н.Б., Назаренко Л.П., Намазова-Баранова Л.С., Неретина А.Ф., Никонова В.С., Орлов А.В., Постников С.С., Протасова Т.А., Семыкин С.Ю., Сергиенко Д.Ф., Симонова О.И., Успенская И.Д., Чернуха М.Ю., Шабалова Л.А., Шагинян И.А., Шерман В.Д.</copyright-holder><copyright-holder xml:lang="en">Amelina Y.L., Asherova I.K., Volkov I.K., Gembitskaya Т.Y., Ginter Y.K., Il’yenkova N.А., Kapranov N.I., Karimova I.P., Kashirskaya N.Y., Kondrat’yeva Y.I., Kostyleva М.N., Krasovsky S.А., Merzlova N.B., Nazarenko L.P., Namazova-Baranova L.S., Neretina А.F., Nikonova V.S., Orlov А.V., Postnikov S.S., Protasova Т.А., Semykin S.Y., Sergienko D.F., Simonova О.I., Uspenskaya I.D., Chernukha М.Y., Shabalova L.А., Shaginyan I.А., Sherman V.D.</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://vsp.spr-journal.ru/jour/article/view/109">https://vsp.spr-journal.ru/jour/article/view/109</self-uri><abstract><p>Ингаляционный путь введения препаратов для лечения поражения легких при муковисцидозе обладает высокой эффективностью. В настоящем согласительном документе (консенсусе) представлены обобщенные данные по ингаляционному применению бронходилататоров, муколитиков, противовоспалительных препаратов, включая глюкокортикоиды, рекомендованных к применению у больных муковисцидозом всех возрастных категорий на территории Российской Федерации.</p></abstract><trans-abstract xml:lang="en"/><kwd-group xml:lang="ru"><kwd>муковисцидоз</kwd><kwd>ингаляционная терапия</kwd><kwd>бронходилататоры</kwd><kwd>муколитики</kwd><kwd>дорназа альфа</kwd><kwd>гипертонический раствор</kwd><kwd>глюкокортикоиды</kwd></kwd-group><kwd-group xml:lang="en"><kwd>cystic fibrosis</kwd><kwd>inhalation therapy</kwd><kwd>bronchodilators</kwd><kwd>mucolytics</kwd><kwd>dornase alfa</kwd><kwd>hypertonic saline</kwd><kwd>glucocorticoids</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">Муковисцидоз. Под ред. Н. И. Капранова, Н. Ю. 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