Modern Approaches in Management of Children with Cystic fibrosis

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 thi...

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Main Authors: Alexander A. Baranov, Leyla S. Namazova-Baranova, Sergey I. Kutsev, Sergey N. Avdeev, Elena V. Polevichenko, Andrey S. Belevskiy, Elena I. Kondratyeva, Olga I. Simonova, Nataliya Yu. Kashirskaya, Victoria D. Sherman, Anna Yu. Voronkova, Evgeniya L. Amelina, Tatyana E. Gembitskaya, Stanislav A. Krasovskiy, Alexey G. Chermenskiy, Tatyana A. Stepanenko, Liliia R. Selimzyanova, Elena A. Vishneva, Yulia V. Gorinova, Elena A. Roslavtseva, Irina K. Asherova, Natalya A. Ilyenkova, Sergey K. Zyryanov, Niso D. Odinayeva, Tatyana Yu. Maksimycheva, Alexander V. Orlov, Sergey Yu. Semykin, Marina Yu. Chernukha, Igor A. Shaginyan, Lusine R. Avetisyan, Galina L. Shumkova, Natalya A. Krylova, Ivan A. Dronov, Maria N. Kostyleva, Ludmila A. Zhelenina, Nikolay N. Klimko, Yuliya V. Borzova, Natalya V. Vasilyeva, Tatyana S. Bogomolova, Anna A. Speranskaya, Irina A. Baranova, Evgeny G. Furman, Vera V. Shadrina, Nikolay F. Shchapov, Nika V. Petrova, Ivan V. Pashkov, Olga M. Tsirulnikova, Dmitriy P. Polyakov, Valeriy M. Svistushkin, Eduard V. Sin'kov, Vyacheslav B. Chernykh, Svetlana A. Repina, Dmitriy A. Blagovidov, Mikhail P. Kostinov, Olga V. Kondratenko, Artem V. Lyamin, Svetlana V. Polikarpova, Alexander V. Polyakov, Tagui A. Adyan, Dmitry V. Goldshtein, Tatiana B. Bukharova, Anna S. Efremova, Elena S. Ovsyankina, Ludmila V. Panova, Irina V. Cherkashina
Format: Article
Language:Russian
Published: Union of pediatricians of Russia 2022-05-01
Series:Педиатрическая фармакология
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Online Access:https://www.pedpharma.ru/jour/article/view/2169
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author Alexander A. Baranov
Leyla S. Namazova-Baranova
Sergey I. Kutsev
Sergey N. Avdeev
Elena V. Polevichenko
Andrey S. Belevskiy
Elena I. Kondratyeva
Olga I. Simonova
Nataliya Yu. Kashirskaya
Victoria D. Sherman
Anna Yu. Voronkova
Evgeniya L. Amelina
Tatyana E. Gembitskaya
Stanislav A. Krasovskiy
Alexey G. Chermenskiy
Tatyana A. Stepanenko
Liliia R. Selimzyanova
Elena A. Vishneva
Yulia V. Gorinova
Elena A. Roslavtseva
Irina K. Asherova
Natalya A. Ilyenkova
Sergey K. Zyryanov
Niso D. Odinayeva
Tatyana Yu. Maksimycheva
Alexander V. Orlov
Sergey Yu. Semykin
Marina Yu. Chernukha
Igor A. Shaginyan
Lusine R. Avetisyan
Galina L. Shumkova
Natalya A. Krylova
Ivan A. Dronov
Maria N. Kostyleva
Ludmila A. Zhelenina
Nikolay N. Klimko
Yuliya V. Borzova
Natalya V. Vasilyeva
Tatyana S. Bogomolova
Anna A. Speranskaya
Irina A. Baranova
Evgeny G. Furman
Vera V. Shadrina
Nikolay F. Shchapov
Nika V. Petrova
Ivan V. Pashkov
Olga M. Tsirulnikova
Dmitriy P. Polyakov
Valeriy M. Svistushkin
Eduard V. Sin'kov
Vyacheslav B. Chernykh
Svetlana A. Repina
Dmitriy A. Blagovidov
Mikhail P. Kostinov
Olga V. Kondratenko
Artem V. Lyamin
Svetlana V. Polikarpova
Alexander V. Polyakov
Tagui A. Adyan
Dmitry V. Goldshtein
Tatiana B. Bukharova
Anna S. Efremova
Elena S. Ovsyankina
Ludmila V. Panova
Irina V. Cherkashina
author_facet Alexander A. Baranov
Leyla S. Namazova-Baranova
Sergey I. Kutsev
Sergey N. Avdeev
Elena V. Polevichenko
Andrey S. Belevskiy
Elena I. Kondratyeva
Olga I. Simonova
Nataliya Yu. Kashirskaya
Victoria D. Sherman
Anna Yu. Voronkova
Evgeniya L. Amelina
Tatyana E. Gembitskaya
Stanislav A. Krasovskiy
Alexey G. Chermenskiy
Tatyana A. Stepanenko
Liliia R. Selimzyanova
Elena A. Vishneva
Yulia V. Gorinova
Elena A. Roslavtseva
Irina K. Asherova
Natalya A. Ilyenkova
Sergey K. Zyryanov
Niso D. Odinayeva
Tatyana Yu. Maksimycheva
Alexander V. Orlov
Sergey Yu. Semykin
Marina Yu. Chernukha
Igor A. Shaginyan
Lusine R. Avetisyan
Galina L. Shumkova
Natalya A. Krylova
Ivan A. Dronov
Maria N. Kostyleva
Ludmila A. Zhelenina
Nikolay N. Klimko
Yuliya V. Borzova
Natalya V. Vasilyeva
Tatyana S. Bogomolova
Anna A. Speranskaya
Irina A. Baranova
Evgeny G. Furman
Vera V. Shadrina
Nikolay F. Shchapov
Nika V. Petrova
Ivan V. Pashkov
Olga M. Tsirulnikova
Dmitriy P. Polyakov
Valeriy M. Svistushkin
Eduard V. Sin'kov
Vyacheslav B. Chernykh
Svetlana A. Repina
Dmitriy A. Blagovidov
Mikhail P. Kostinov
Olga V. Kondratenko
Artem V. Lyamin
Svetlana V. Polikarpova
Alexander V. Polyakov
Tagui A. Adyan
Dmitry V. Goldshtein
Tatiana B. Bukharova
Anna S. Efremova
Elena S. Ovsyankina
Ludmila V. Panova
Irina V. Cherkashina
author_sort Alexander A. Baranov
collection DOAJ
description 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.
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spelling doaj-art-143f4fcbf4674dee93deee9bc716f4412025-08-20T03:19:39ZrusUnion of pediatricians of RussiaПедиатрическая фармакология1727-57762500-30892022-05-0119215319510.15690/pf.v19i2.24171904Modern Approaches in Management of Children with Cystic fibrosisAlexander A. Baranov0Leyla S. Namazova-Baranova1Sergey I. Kutsev2Sergey N. Avdeev3Elena V. Polevichenko4Andrey S. Belevskiy5Elena I. Kondratyeva6Olga I. Simonova7Nataliya Yu. Kashirskaya8Victoria D. Sherman9Anna Yu. Voronkova10Evgeniya L. Amelina11Tatyana E. Gembitskaya12Stanislav A. Krasovskiy13Alexey G. Chermenskiy14Tatyana A. Stepanenko15Liliia R. Selimzyanova16Elena A. Vishneva17Yulia V. Gorinova18Elena A. Roslavtseva19Irina K. Asherova20Natalya A. Ilyenkova21Sergey K. Zyryanov22Niso D. Odinayeva23Tatyana Yu. Maksimycheva24Alexander V. Orlov25Sergey Yu. Semykin26Marina Yu. Chernukha27Igor A. Shaginyan28Lusine R. Avetisyan29Galina L. Shumkova30Natalya A. Krylova31Ivan A. Dronov32Maria N. Kostyleva33Ludmila A. Zhelenina34Nikolay N. Klimko35Yuliya V. Borzova36Natalya V. Vasilyeva37Tatyana S. Bogomolova38Anna A. Speranskaya39Irina A. Baranova40Evgeny G. Furman41Vera V. Shadrina42Nikolay F. Shchapov43Nika V. Petrova44Ivan V. Pashkov45Olga M. Tsirulnikova46Dmitriy P. Polyakov47Valeriy M. Svistushkin48Eduard V. Sin'kov49Vyacheslav B. Chernykh50Svetlana A. Repina51Dmitriy A. Blagovidov52Mikhail P. Kostinov53Olga V. Kondratenko54Artem V. Lyamin55Svetlana V. Polikarpova56Alexander V. Polyakov57Tagui A. Adyan58Dmitry V. Goldshtein59Tatiana B. Bukharova60Anna S. Efremova61Elena S. Ovsyankina62Ludmila V. Panova63Irina V. Cherkashina64Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Sechenov First Moscow State Medical UniversityResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical University; Belgorod National Research UniversityResearch Centre for Medical GeneticsSechenov First Moscow State Medical University; Pulmonology Research Institute of Federal Medico-Biological Agency of RussiaSechenov First Moscow State Medical UniversityPirogov Russian National Research Medical UniversityResearch Centre for Medical Genetics; Research Clinical Institute of Childhood of the Ministry of Health of Moscow RegionSechenov First Moscow State Medical University; National Medical Research Center of Children’s Health; Morozov Children’s City HospitalResearch Centre for Medical GeneticsResearch Centre for Medical GeneticsResearch Centre for Medical GeneticsResearch Centre for Medical Genetics; Pulmonology Research Institute of Federal Medico-Biological Agency of RussiaPavlov First Saint Petersburg State Medical UniversityResearch Centre for Medical Genetics; Pulmonology Research Institute of Federal Medico-Biological Agency of RussiaPavlov First Saint Petersburg State Medical UniversityCity Multidisciplinary Hospital № 2Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Sechenov First Moscow State Medical UniversityResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityNational Medical Research Center of Children’s HealthNational Medical Research Center of Children’s HealthCentral Clinical HospitalKrasnoyarsk State Medical University named after prof. V.F. Voino-YasenetskyRUDN UniversityResearch Clinical Institute of Childhood of the Ministry of Health of Moscow RegionResearch Centre for Medical GeneticsNorth-Western State Medical University named after I.I. Mechnikov; St. Olga Children’s City HospitalRussian Children’s Clinical HospitalNational Research Center for Epidemiology and Microbiology named after Honorary Academician N.F. GamaleyaNational Research Center for Epidemiology and Microbiology named after Honorary Academician N.F. GamaleyaNational Research Center for Epidemiology and Microbiology named after Honorary Academician N.F. GamaleyaPulmonology Research Institute of Federal Medico-Biological Agency of RussiaPulmonology Research Institute of Federal Medico-Biological Agency of RussiaSechenov First Moscow State Medical University; Research Clinical Institute of Childhood of the Ministry of Health of Moscow RegionPirogov Russian National Research Medical University; Russian Children’s Clinical HospitalSaint Petersburg State Pediatric Medical UniversityNorth-Western State Medical University named after I.I. MechnikovNorth-Western State Medical University named after I.I. MechnikovNorth-Western State Medical University named after I.I. Mechnikov; Kashkin Research Institute of Medical MycologyNorth-Western State Medical University named after I.I. MechnikovPavlov First Saint Petersburg State Medical UniversityPirogov Russian National Research Medical UniversityPerm State Medical University named after academician E.A. WagnerPerm State Medical University named after academician E.A. WagnerMoscow Regional Center for Maternity and Childhood ProtectionResearch Centre for Medical GeneticsV.I. Shumakov National Medical Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of Russian FederationSechenov First Moscow State Medical UniversityPirogov Russian National Research Medical University; National Medical Research Center for Otorhinolaryngology of Federal Medico-Biological Agency of RussiaSechenov First Moscow State Medical UniversitySechenov First Moscow State Medical UniversityResearch Centre for Medical GeneticsResearch Centre for Medical GeneticsPirogov Russian National Research Medical University; I.I. Mechnikov Research Institute of Vaccines and SeraI.I. Mechnikov Research Institute of Vaccines and SeraSamara State Medical UniversitySamara State Medical UniversityCity Clinical Hospital № 15 named after O.M. FilatovResearch Centre for Medical GeneticsPirogov Russian National Research Medical University; Research Centre for Medical GeneticsResearch Centre for Medical GeneticsResearch Centre for Medical GeneticsResearch Centre for Medical GeneticsCentral Research Institute of TuberculosisCentral Research Institute of TuberculosisChildren’s Research and Clinical Center for Infectious Diseases of Federal Medico-Biological Agency of RussiaThe 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.https://www.pedpharma.ru/jour/article/view/2169cystic fibrosisdiagnosistreatmentkinesitherapyenzyme replacement therapychildren
spellingShingle Alexander A. Baranov
Leyla S. Namazova-Baranova
Sergey I. Kutsev
Sergey N. Avdeev
Elena V. Polevichenko
Andrey S. Belevskiy
Elena I. Kondratyeva
Olga I. Simonova
Nataliya Yu. Kashirskaya
Victoria D. Sherman
Anna Yu. Voronkova
Evgeniya L. Amelina
Tatyana E. Gembitskaya
Stanislav A. Krasovskiy
Alexey G. Chermenskiy
Tatyana A. Stepanenko
Liliia R. Selimzyanova
Elena A. Vishneva
Yulia V. Gorinova
Elena A. Roslavtseva
Irina K. Asherova
Natalya A. Ilyenkova
Sergey K. Zyryanov
Niso D. Odinayeva
Tatyana Yu. Maksimycheva
Alexander V. Orlov
Sergey Yu. Semykin
Marina Yu. Chernukha
Igor A. Shaginyan
Lusine R. Avetisyan
Galina L. Shumkova
Natalya A. Krylova
Ivan A. Dronov
Maria N. Kostyleva
Ludmila A. Zhelenina
Nikolay N. Klimko
Yuliya V. Borzova
Natalya V. Vasilyeva
Tatyana S. Bogomolova
Anna A. Speranskaya
Irina A. Baranova
Evgeny G. Furman
Vera V. Shadrina
Nikolay F. Shchapov
Nika V. Petrova
Ivan V. Pashkov
Olga M. Tsirulnikova
Dmitriy P. Polyakov
Valeriy M. Svistushkin
Eduard V. Sin'kov
Vyacheslav B. Chernykh
Svetlana A. Repina
Dmitriy A. Blagovidov
Mikhail P. Kostinov
Olga V. Kondratenko
Artem V. Lyamin
Svetlana V. Polikarpova
Alexander V. Polyakov
Tagui A. Adyan
Dmitry V. Goldshtein
Tatiana B. Bukharova
Anna S. Efremova
Elena S. Ovsyankina
Ludmila V. Panova
Irina V. Cherkashina
Modern Approaches in Management of Children with Cystic fibrosis
Педиатрическая фармакология
cystic fibrosis
diagnosis
treatment
kinesitherapy
enzyme replacement therapy
children
title Modern Approaches in Management of Children with Cystic fibrosis
title_full Modern Approaches in Management of Children with Cystic fibrosis
title_fullStr Modern Approaches in Management of Children with Cystic fibrosis
title_full_unstemmed Modern Approaches in Management of Children with Cystic fibrosis
title_short Modern Approaches in Management of Children with Cystic fibrosis
title_sort modern approaches in management of children with cystic fibrosis
topic cystic fibrosis
diagnosis
treatment
kinesitherapy
enzyme replacement therapy
children
url https://www.pedpharma.ru/jour/article/view/2169
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