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ppharm

Педиатрическая фармакология

Pediatric pharmacology

1727-57762500-3089

Издательство «ПедиатрЪ»

10.15690/pf.v19i2.2417

ppharm-2169

Research Article

КЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИ

CLINICAL RECOMMENDATIONS

Современные подходы к ведению детей с муковисцидозом

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НИИ педиатрии и охраны здоровья детей НКЦ №2 РНЦХ им. акад. Б.В. Петровского; Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)РоссияResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Sechenov First Moscow State Medical UniversityRussian Federation

НИИ педиатрии и охраны здоровья детей НКЦ №2 ФГБНУ «РНЦХ им. акад. Б.В. Петровского; Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Белгородский государственный национальный исследовательский университетРоссияResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical University; Belgorod National Research UniversityRussian Federation

Медико-генетический научный центр им. академика Н.П. БочковаРоссияResearch Centre for Medical GeneticsRussian Federation

Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет); Научно-исследовательский институт пульмонологии ФМБАРоссияSechenov First Moscow State Medical University; Pulmonology Research Institute of Federal Medico-Biological Agency of RussiaRussian Federation

Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)РоссияSechenov First Moscow State Medical UniversityRussian Federation

Российский национальный исследовательский медицинский университет им. Н.И. ПироговаРоссияPirogov Russian National Research Medical UniversityRussian Federation

Медико-генетический научный центр им. академика Н.П. Бочкова; Научно-исследовательский клинический институт детства Министерства здравоохранения Московской областиРоссияResearch Centre for Medical Genetics; Research Clinical Institute of Childhood of the Ministry of Health of Moscow RegionRussian Federation

Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет); Национальный медицинский исследовательский центр здоровья детей; Морозовская детская городская клиническая больница ДЗМРоссияSechenov First Moscow State Medical University; National Medical Research Center of Children’s Health; Morozov Children’s City HospitalRussian Federation

Медико-генетический научный центр им. академика Н.П. Бочкова; Научно-исследовательский институт пульмонологии ФМБАРоссияResearch Centre for Medical Genetics; Pulmonology Research Institute of Federal Medico-Biological Agency of RussiaRussian Federation

Первый Санкт-Петербургский государственный медицинский университет им. И.П. ПавловаРоссияPavlov First Saint Petersburg State Medical UniversityRussian Federation

Городская многопрофильная больница № 2РоссияCity Multidisciplinary Hospital № 2Russian Federation

НИИ педиатрии и охраны здоровья детей НКЦ №2 ФГБНУ «РНЦХ им. акад. Б.В. Петровского; Российский национальный исследовательский медицинский университет им. Н.И. ПироговаРоссияResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityRussian Federation

Национальный медицинский исследовательский центр здоровья детейРоссияNational Medical Research Center of Children’s HealthRussian Federation

Центральная городская больницаРоссияCentral Clinical HospitalRussian Federation

Красноярский государственный медицинский университет им. проф. В.Ф. Войно-ЯсенецкогоРоссияKrasnoyarsk State Medical University named after prof. V.F. Voino-YasenetskyRussian Federation

Российский университет дружбы народовРоссияRUDN UniversityRussian Federation

Научно-исследовательский клинический институт детства Министерства здравоохранения Московской областиРоссияResearch Clinical Institute of Childhood of the Ministry of Health of Moscow RegionRussian Federation

Северо-Западный государственный медицинский университет им. И.И. Мечникова; Детская городская больница Святой ОльгиРоссияNorth-Western State Medical University named after I.I. Mechnikov; St. Olga Children’s City HospitalRussian Federation

Российская детская клиническая больницаРоссияRussian Children’s Clinical HospitalRussian Federation

Национальный исследовательский центр эпидемиологии и микробиологии имени почетного академика Н.Ф. ГамалеиРоссияNational Research Center for Epidemiology and Microbiology named after Honorary Academician N.F. GamaleyaRussian Federation

Научно-исследовательский институт пульмонологии ФМБАРоссияPulmonology Research Institute of Federal Medico-Biological Agency of RussiaRussian Federation

Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет); Научно-исследовательский клинический институт детства Министерства здравоохранения Московской областиРоссияSechenov First Moscow State Medical University; Research Clinical Institute of Childhood of the Ministry of Health of Moscow RegionRussian Federation

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Российская детская клиническая больницаРоссияPirogov Russian National Research Medical University; Russian Children’s Clinical HospitalRussian Federation

Санкт-Петербургский государственный педиатрический медицинский университетРоссияSaint Petersburg State Pediatric Medical UniversityRussian Federation

Северо-Западный государственный медицинский университет им. И.И. МечниковаРоссияNorth-Western State Medical University named after I.I. MechnikovRussian Federation

Северо-Западный государственный медицинский университет им. И.И. Мечникова; НИИ медицинской микологии им. П.Н. КашкинаРоссияNorth-Western State Medical University named after I.I. Mechnikov; Kashkin Research Institute of Medical MycologyRussian Federation

Пермский государственный медицинский университет им. академика Е. А. ВагнераРоссияPerm State Medical University named after academician E.A. WagnerRussian Federation

Московский областной центр охраны материнства и детстваРоссияMoscow Regional Center for Maternity and Childhood ProtectionRussian Federation

Национальный медицинский исследовательский центр трансплантологии и искусственных органов имени академика В.И. ШумаковаРоссияV.I. Shumakov National Medical Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of Russian FederationRussian Federation

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Национальный медицинский исследовательский центр оториноларингологии ФМБАРоссияPirogov Russian National Research Medical University; National Medical Research Center for Otorhinolaryngology of Federal Medico-Biological Agency of RussiaRussian Federation

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; НИИ вакцин и сывороток им. И.И. МечниковаРоссияPirogov Russian National Research Medical University; I.I. Mechnikov Research Institute of Vaccines and SeraRussian Federation

НИИ вакцин и сывороток им. И.И. МечниковаРоссияI.I. Mechnikov Research Institute of Vaccines and SeraRussian Federation

Самарский государственный медицинский университетРоссияSamara State Medical UniversityRussian Federation

Городская клиническая больница № 15 им. О.М. ФилатоваРоссияCity Clinical Hospital № 15 named after O.M. FilatovRussian Federation

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Медико-генетический научный центр им. академика Н.П. БочковаРоссияPirogov Russian National Research Medical University; Research Centre for Medical GeneticsRussian Federation

Центральный НИИ туберкулезаРоссияCentral Research Institute of TuberculosisRussian Federation

Детский научно-клинический центр инфекционных болезней ФМБА РоссииРоссияChildren’s Research and Clinical Center for Infectious Diseases of Federal Medico-Biological Agency of RussiaRussian Federation

2022

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Copyright © Баранов А.А., Намазова-Баранова Л.С., Куцев С.И., Авдеев С.Н., Полевиченко Е.В., Белевский А.С., Кондратьева Е.И., Симонова О.И., Каширская Н.Ю., Шерман В.Д., Воронкова А.Ю., Амелина Е.Л., Гембицкая Т.Е., Красовский С.А., Черменский А.Г., Степаненко Т.А., Селимзянова Л.Р., Вишнева Е.А., Горинова Ю.В., Рославцева Е.А., Ашерова И.К., Ильенкова Н.А., Зырянов С.К., Одинаева Н.Д., Максимычева Т.Ю., Орлов А.В., Семыкин С.Ю., Чернуха М.Ю., Шагинян И.А., Аветисян Л.Р., Шумкова Г.Л., Крылова Н.А., Дронов И.А., Костылева М.Н., Желенина Л.А., Климко Н.Н., Борзова Ю.В., Васильева Н.В., Богомолова Т.С., Сперанская А.А., Баранова И.А., Фурман Е.Г., Шадрина В.В., Щапов Н.Ф., Петрова Н.В., Пашков И.В., Цирульникова О.М., Поляков Д.П., Свистушкин В.М., Синьков Э.В., Черных В.Б., Репина С.А., Благовидов Д.А., Костинов М.П., Кондратенко О.В., Лямин А.В., Поликарпова С.В., Поляков А.В., Адян Т.А., Гольдштейн Д.В., Бухарова Т.Б., Ефремова А.С., Овсянкина Е.С., Панова Л.В., Черкашина И.В., 2022

2022

Баранов А.А., Намазова-Баранова Л.С., Куцев С.И., Авдеев С.Н., Полевиченко Е.В., Белевский А.С., Кондратьева Е.И., Симонова О.И., Каширская Н.Ю., Шерман В.Д., Воронкова А.Ю., Амелина Е.Л., Гембицкая Т.Е., Красовский С.А., Черменский А.Г., Степаненко Т.А., Селимзянова Л.Р., Вишнева Е.А., Горинова Ю.В., Рославцева Е.А., Ашерова И.К., Ильенкова Н.А., Зырянов С.К., Одинаева Н.Д., Максимычева Т.Ю., Орлов А.В., Семыкин С.Ю., Чернуха М.Ю., Шагинян И.А., Аветисян Л.Р., Шумкова Г.Л., Крылова Н.А., Дронов И.А., Костылева М.Н., Желенина Л.А., Климко Н.Н., Борзова Ю.В., Васильева Н.В., Богомолова Т.С., Сперанская А.А., Баранова И.А., Фурман Е.Г., Шадрина В.В., Щапов Н.Ф., Петрова Н.В., Пашков И.В., Цирульникова О.М., Поляков Д.П., Свистушкин В.М., Синьков Э.В., Черных В.Б., Репина С.А., Благовидов Д.А., Костинов М.П., Кондратенко О.В., Лямин А.В., Поликарпова С.В., Поляков А.В., Адян Т.А., Гольдштейн Д.В., Бухарова Т.Б., Ефремова А.С., Овсянкина Е.С., Панова Л.В., Черкашина И.В.

Baranov A.A., Namazova-Baranova L.S., Kutsev S.I., Avdeev S.N., Polevichenko E.V., Belevskiy A.S., Kondratyeva E.I., Simonova O.I., Kashirskaya N.Y., Sherman V.D., Voronkova A.Y., Amelina E.L., Gembitskaya T.E., Krasovskiy S.A., Chermenskiy A.G., Stepanenko T.A., Selimzyanova L.R., Vishneva E.A., Gorinova Y.V., Roslavtseva E.A., Asherova I.K., Ilyenkova N.A., Zyryanov S.K., Odinayeva N.D., Maksimycheva T.Y., Orlov A.V., Semykin S.Y., Chernukha M.Y., Shaginyan I.A., Avetisyan L.R., Shumkova G.L., Krylova N.A., Dronov I.A., Kostyleva M.N., Zhelenina L.A., Klimko N.N., Borzova Y.V., Vasilyeva N.V., Bogomolova T.S., Speranskaya A.A., Baranova I.A., Furman E.G., Shadrina V.V., Shchapov N.F., Petrova N.V., Pashkov I.V., Tsirulnikova O.M., Polyakov D.P., Svistushkin V.M., Sin'kov E.V., Chernykh V.B., Repina S.A., Blagovidov D.A., Kostinov M.P., Kondratenko O.V., Lyamin A.V., Polikarpova S.V., Polyakov A.V., Adyan T.A., Goldshtein D.V., Bukharova T.B., Efremova A.S., Ovsyankina E.S., Panova L.V., Cherkashina I.V.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://www.pedpharma.ru/jour/article/view/2169

Проблема своевременной диагностики и правильного ведения пациентов с муковисцидозом актуальна не только в нашей стране, но и во всем мире. Экспертами Союза педиатров России с современных позиций рассмотрены проблемы этиологии, патогенеза, эпидемиологии, диагностики и лечения этого генетического заболевания. Особое внимание уделено методам скрининга для раннего выявления муковисцидоза. Обоснованы принципы комплексной терапии, в том числе рационального использования антибактериальных, муколитических препаратов и ферментозаместительной терапии, во многом определяющие прогноз болезни.

The problem of timely diagnosis and proper management of patients with cystic fibrosis is crucial not only in our country, but throughout the world. Experts of the Union of Pediatricians of Russia have considered various issues of etiology, pathogenesis, epidemiology, diagnosis, and treatment of this genetic disease in a modern light. Particular attention was paid to screening methods for early diagnosis of cystic fibrosis. The principles of complex therapy were justified, including rational use of antibacterial and mucolytic drugs and enzyme replacement therapy that significantly determine the disease prognosis.

муковисцидоздиагностикалечениекинезитерапияферментозаместительная терапиядети

cystic fibrosisdiagnosistreatmentkinesitherapyenzyme replacement therapychildren

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The authors declare that there are no conflicts of interest present.