Педиатрическая фармакология (May 2022)
Modern Approaches in Management of Children with Cystic fibrosis
- 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
Affiliations
- Alexander A. Baranov
- Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Sechenov First Moscow State Medical University
- Leyla S. Namazova-Baranova
- Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical University; Belgorod National Research University
- Sergey I. Kutsev
- Research Centre for Medical Genetics
- Sergey N. Avdeev
- Sechenov First Moscow State Medical University; Pulmonology Research Institute of Federal Medico-Biological Agency of Russia
- Elena V. Polevichenko
- Sechenov First Moscow State Medical University
- Andrey S. Belevskiy
- Pirogov Russian National Research Medical University
- Elena I. Kondratyeva
- Research Centre for Medical Genetics; Research Clinical Institute of Childhood of the Ministry of Health of Moscow Region
- Olga I. Simonova
- Sechenov First Moscow State Medical University; National Medical Research Center of Children’s Health; Morozov Children’s City Hospital
- Nataliya Yu. Kashirskaya
- Research Centre for Medical Genetics
- Victoria D. Sherman
- Research Centre for Medical Genetics
- Anna Yu. Voronkova
- Research Centre for Medical Genetics
- Evgeniya L. Amelina
- Research Centre for Medical Genetics; Pulmonology Research Institute of Federal Medico-Biological Agency of Russia
- Tatyana E. Gembitskaya
- Pavlov First Saint Petersburg State Medical University
- Stanislav A. Krasovskiy
- Research Centre for Medical Genetics; Pulmonology Research Institute of Federal Medico-Biological Agency of Russia
- Alexey G. Chermenskiy
- Pavlov First Saint Petersburg State Medical University
- Tatyana A. Stepanenko
- City Multidisciplinary Hospital № 2
- Liliia R. Selimzyanova
- Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Sechenov First Moscow State Medical University
- Elena A. Vishneva
- Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical University
- Yulia V. Gorinova
- National Medical Research Center of Children’s Health
- Elena A. Roslavtseva
- National Medical Research Center of Children’s Health
- Irina K. Asherova
- Central Clinical Hospital
- Natalya A. Ilyenkova
- Krasnoyarsk State Medical University named after prof. V.F. Voino-Yasenetsky
- Sergey K. Zyryanov
- RUDN University
- Niso D. Odinayeva
- Research Clinical Institute of Childhood of the Ministry of Health of Moscow Region
- Tatyana Yu. Maksimycheva
- Research Centre for Medical Genetics
- Alexander V. Orlov
- North-Western State Medical University named after I.I. Mechnikov; St. Olga Children’s City Hospital
- Sergey Yu. Semykin
- Russian Children’s Clinical Hospital
- Marina Yu. Chernukha
- National Research Center for Epidemiology and Microbiology named after Honorary Academician N.F. Gamaleya
- Igor A. Shaginyan
- National Research Center for Epidemiology and Microbiology named after Honorary Academician N.F. Gamaleya
- Lusine R. Avetisyan
- National Research Center for Epidemiology and Microbiology named after Honorary Academician N.F. Gamaleya
- Galina L. Shumkova
- Pulmonology Research Institute of Federal Medico-Biological Agency of Russia
- Natalya A. Krylova
- Pulmonology Research Institute of Federal Medico-Biological Agency of Russia
- Ivan A. Dronov
- Sechenov First Moscow State Medical University; Research Clinical Institute of Childhood of the Ministry of Health of Moscow Region
- Maria N. Kostyleva
- Pirogov Russian National Research Medical University; Russian Children’s Clinical Hospital
- Ludmila A. Zhelenina
- Saint Petersburg State Pediatric Medical University
- Nikolay N. Klimko
- North-Western State Medical University named after I.I. Mechnikov
- Yuliya V. Borzova
- North-Western State Medical University named after I.I. Mechnikov
- Natalya V. Vasilyeva
- North-Western State Medical University named after I.I. Mechnikov; Kashkin Research Institute of Medical Mycology
- Tatyana S. Bogomolova
- North-Western State Medical University named after I.I. Mechnikov
- Anna A. Speranskaya
- Pavlov First Saint Petersburg State Medical University
- Irina A. Baranova
- Pirogov Russian National Research Medical University
- Evgeny G. Furman
- Perm State Medical University named after academician E.A. Wagner
- Vera V. Shadrina
- Perm State Medical University named after academician E.A. Wagner
- Nikolay F. Shchapov
- Moscow Regional Center for Maternity and Childhood Protection
- Nika V. Petrova
- Research Centre for Medical Genetics
- Ivan V. Pashkov
- V.I. Shumakov National Medical Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of Russian Federation
- Olga M. Tsirulnikova
- Sechenov First Moscow State Medical University
- Dmitriy P. Polyakov
- Pirogov Russian National Research Medical University; National Medical Research Center for Otorhinolaryngology of Federal Medico-Biological Agency of Russia
- Valeriy M. Svistushkin
- Sechenov First Moscow State Medical University
- Eduard V. Sin'kov
- Sechenov First Moscow State Medical University
- Vyacheslav B. Chernykh
- Research Centre for Medical Genetics
- Svetlana A. Repina
- Research Centre for Medical Genetics
- Dmitriy A. Blagovidov
- Pirogov Russian National Research Medical University; I.I. Mechnikov Research Institute of Vaccines and Sera
- Mikhail P. Kostinov
- I.I. Mechnikov Research Institute of Vaccines and Sera
- Olga V. Kondratenko
- Samara State Medical University
- Artem V. Lyamin
- Samara State Medical University
- Svetlana V. Polikarpova
- City Clinical Hospital № 15 named after O.M. Filatov
- Alexander V. Polyakov
- Research Centre for Medical Genetics
- Tagui A. Adyan
- Pirogov Russian National Research Medical University; Research Centre for Medical Genetics
- Dmitry V. Goldshtein
- Research Centre for Medical Genetics
- Tatiana B. Bukharova
- Research Centre for Medical Genetics
- Anna S. Efremova
- Research Centre for Medical Genetics
- Elena S. Ovsyankina
- Central Research Institute of Tuberculosis
- Ludmila V. Panova
- Central Research Institute of Tuberculosis
- Irina V. Cherkashina
- Children’s Research and Clinical Center for Infectious Diseases of Federal Medico-Biological Agency of Russia
- DOI
- https://doi.org/10.15690/pf.v19i2.2417
- Journal volume & issue
-
Vol. 19,
no. 2
pp. 153 – 195
Abstract
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.
Keywords
