Modern Approaches in Management of Children with Cystic fibrosis-Reference-Cited by-同舟云学术

Modern Approaches in Management of Children with Cystic fibrosis

Published:2022-05-27 Issue:2 Volume:19 Page:153-195
ISSN:2500-3089
Container-title:Pediatric pharmacology
language:
Short-container-title:Pediatr. farmakol.

Author:

Baranov Alexander A.¹^ORCID,Namazova-Baranova Leyla S.²^ORCID,Kutsev Sergey I.³^ORCID,Avdeev Sergey N.⁴^ORCID,Polevichenko Elena V.⁵,Belevskiy Andrey S.⁶^ORCID,Kondratyeva Elena I.⁷^ORCID,Simonova Olga I.⁸^ORCID,Kashirskaya Nataliya Yu.³^ORCID,Sherman Victoria D.³^ORCID,Voronkova Anna Yu.³^ORCID,Amelina Evgeniya L.⁹^ORCID,Gembitskaya Tatyana E.¹⁰^ORCID,Krasovskiy Stanislav A.⁹^ORCID,Chermenskiy Alexey G.¹⁰^ORCID,Stepanenko Tatyana A.¹¹^ORCID,Selimzyanova Liliia R.¹^ORCID,Vishneva Elena A.¹²^ORCID,Gorinova Yulia V.¹³^ORCID,Roslavtseva Elena A.¹³^ORCID,Asherova Irina K.¹⁴,Ilyenkova Natalya A.¹⁵^ORCID,Zyryanov Sergey K.¹⁶^ORCID,Odinayeva Niso D.¹⁷^ORCID,Maksimycheva Tatyana Yu.³^ORCID,Orlov Alexander V.¹⁸,Semykin Sergey Yu.¹⁹^ORCID,Chernukha Marina Yu.²⁰^ORCID,Shaginyan Igor A.²⁰^ORCID,Avetisyan Lusine R.²⁰^ORCID,Shumkova Galina L.²¹^ORCID,Krylova Natalya A.²¹^ORCID,Dronov Ivan A.²²^ORCID,Kostyleva Maria N.²³^ORCID,Zhelenina Ludmila A.²⁴,Klimko Nikolay N.²⁵^ORCID,Borzova Yuliya V.²⁵^ORCID,Vasilyeva Natalya V.²⁶^ORCID,Bogomolova Tatyana S.²⁵^ORCID,Speranskaya Anna A.¹⁰^ORCID,Baranova Irina A.⁶^ORCID,Furman Evgeny G.²⁷^ORCID,Shadrina Vera V.²⁷^ORCID,Shchapov Nikolay F.²⁸,Petrova Nika V.³^ORCID,Pashkov Ivan V.²⁹,Tsirulnikova Olga M.⁵^ORCID,Polyakov Dmitriy P.³⁰^ORCID,Svistushkin Valeriy M.⁵^ORCID,Sin'kov Eduard V.⁵^ORCID,Chernykh Vyacheslav B.³^ORCID,Repina Svetlana A.³^ORCID,Blagovidov Dmitriy A.³¹,Kostinov Mikhail P.³²^ORCID,Kondratenko Olga V.³³^ORCID,Lyamin Artem V.³³^ORCID,Polikarpova Svetlana V.³⁴^ORCID,Polyakov Alexander V.³^ORCID,Adyan Tagui A.³⁵^ORCID,Goldshtein Dmitry V.³^ORCID,Bukharova Tatiana B.³^ORCID,Efremova Anna S.³^ORCID,Ovsyankina Elena S.³⁶^ORCID,Panova Ludmila V.³⁶^ORCID,Cherkashina Irina V.³⁷^ORCID

Affiliation:

1. Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Sechenov First Moscow State Medical University

2. 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

3. Research Centre for Medical Genetics

4. Sechenov First Moscow State Medical University; Pulmonology Research Institute of Federal Medico-Biological Agency of Russia

5. Sechenov First Moscow State Medical University

6. Pirogov Russian National Research Medical University

7. Research Centre for Medical Genetics; Research Clinical Institute of Childhood of the Ministry of Health of Moscow Region

8. Sechenov First Moscow State Medical University; National Medical Research Center of Children’s Health; Morozov Children’s City Hospital

9. Research Centre for Medical Genetics; Pulmonology Research Institute of Federal Medico-Biological Agency of Russia

10. Pavlov First Saint Petersburg State Medical University

11. City Multidisciplinary Hospital № 2

12. Research Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical University

13. National Medical Research Center of Children’s Health

14. Central Clinical Hospital

15. Krasnoyarsk State Medical University named after prof. V.F. Voino-Yasenetsky

16. RUDN University

17. Research Clinical Institute of Childhood of the Ministry of Health of Moscow Region

18. North-Western State Medical University named after I.I. Mechnikov; St. Olga Children’s City Hospital

19. Russian Children’s Clinical Hospital

20. National Research Center for Epidemiology and Microbiology named after Honorary Academician N.F. Gamaleya

21. Pulmonology Research Institute of Federal Medico-Biological Agency of Russia

22. Sechenov First Moscow State Medical University; Research Clinical Institute of Childhood of the Ministry of Health of Moscow Region

23. Pirogov Russian National Research Medical University; Russian Children’s Clinical Hospital

24. Saint Petersburg State Pediatric Medical University

25. North-Western State Medical University named after I.I. Mechnikov

26. North-Western State Medical University named after I.I. Mechnikov; Kashkin Research Institute of Medical Mycology

27. Perm State Medical University named after academician E.A. Wagner

28. Moscow Regional Center for Maternity and Childhood Protection

29. V.I. Shumakov National Medical Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of Russian Federation

30. Pirogov Russian National Research Medical University; National Medical Research Center for Otorhinolaryngology of Federal Medico-Biological Agency of Russia

31. Pirogov Russian National Research Medical University; I.I. Mechnikov Research Institute of Vaccines and Sera

32. I.I. Mechnikov Research Institute of Vaccines and Sera

33. Samara State Medical University

34. City Clinical Hospital № 15 named after O.M. Filatov

35. Pirogov Russian National Research Medical University; Research Centre for Medical Genetics

36. Central Research Institute of Tuberculosis

37. Children’s Research and Clinical Center for Infectious Diseases of Federal Medico-Biological Agency of Russia

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.

Publisher

Paediatrician Publishers LLC

Subject

Pulmonary and Respiratory Medicine,Pediatrics, Perinatology and Child Health

Reference249 articles.

1. Mukoviscidoz. Kapranov NI, Kashirskaja NJ, eds. Moscow: MEDPRAKTIKA-M; 2014. 672 p. (In Russ).

2. Nacional'nyj konsensus “Mukoviscidoz: opredele-nie, diagnosticheskie kriterii, terapija”. Kondrat'eva EI, Kashirskaja NJu, Kapranov NI, eds. Moscow: OOO “Kompanija BORGES”; 2016. 205 p. (In Russ).] Доступно по: https://mukoviscidoz.org/doc/konsensus/CF_consensus_2017.pdf. Ссылка активна на 17.04.2022.

3. De Boeck K. Cystic fibrosis in the year 2020: disease with a new face. Acta Paediatr. 2020;109(5):893-899. doi: https://doi.org/10.1111/apa.15155

4. Lopes-Pacheco M. CFTR modulators: Shedding light on precision medicine for cystic fibrosis. Front Pharmacol. 2016;7:275. doi: https://doi.org/10.3389/fphar.2016.00275

5. Registr bol'nyh mukoviscidozom v Rossijskoj Federacii. 2019 god. Voronkova AJu, Amelina EL, Kashirskaja NJu, et al. Moscow: ID “MEDPRAKTIKA-M”; 2021. — 68 p. (In Russ). Доступно по: https://mukoviscidoz.org/doc/registr/10472_block_Registre_2017%20site.pdf. Ссылка активна на 17.04.2022.

Cited by 4 articles. 订阅此论文施引文献订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献

1. The Risk of Developing of Malnutrition and the Principles of Correction of Nutritional Status Disorders in Children with Cystic Fibrosis;Pediatric pharmacology;2023-11-17

2. Prevalence of antimicrobial-resistant Pseudomonas aeruginosa strains isolated from patients with cystic fibrosis;Meditsinskiy sovet = Medical Council;2023-11-17

3. Method for the prevention of hospital infection in Saint Petersburg;Medical academic journal;2022-11-06

4. Influence of exogenous and endogenous factors on lung function in children and adolescents with cysistic fibriosis with genetic variant F508DEL in homozygous state;Meditsinskiy sovet = Medical Council;2022-10-15