Review of clinical guidelines “Cystic fibrosis”, 2020
E. I. Kondratyeva,
E. L. Amelina,
M. Yu. Chernukha,
V. D. Sherman,
S. A. Krasovskiy,
N. Yu. Kashirskaya,
O. I. Simonova,
S. N. Avdeev,
L. S. Namazova-Baranova,
T. E. Gembitskaya,
S. I. Kutsev https://doi.org/10.18093/0869-0189-2021-31-2-135-146
Abstract
The aim of the experts who provide health care for patients with cystic fibrosis (CF) has been to develop clinical guidelines on cystic fibrosis for children and adults.
Methods. The 2020 Clinical Guidelines were based on the European consensus documents on cystic fibrosis and the national consensus “Cystic fibrosis: definition, diagnostic criteria, therapy” (2016, 2019). When creating clinical guidelines, the experts were guided by Article 2 of the Federal Law No.323-FL “On the basics of health care in the Russian Federation” as of November 21st, 2011, and the regulatory legal documents governing the development and approval of clinical guidelines (https://rosmedex.ru/kr/).
Results. The clinical guidelines were approved by the Federal State Budgetary Institution “Centre for Expertise and Quality Control of Medical Care” of the Russian Ministry of Health in August 2020. Standards of medical care for children and adults with cystic fibrosis were prepared based on these guidelines. The diagnostic algorithms have been expanded with a three-stage DNA diagnostic strategy, functional assays of CFTR channel, laboratory and instrumental diagnostic tools for assessing disorders of respiratory and other systems, diagnosis of the microbial inflammatory process, microbiological monitoring, and therapeutic approaches. The antibiotic therapy with dosages and courses is described in detail. The updated document should describe a unified tactic for the management of Russian CF patients.
Conclusion. Unified clinical guidelines for children and adults should develop unified tactics for the diagnosis, therapy, and follow-up of CF patients in the Russian Federation and ensure continuity between health care services for children and adults.
Keywords
Supporting agencies: Zambon Pharma LLC provided financial support for this publication. Zambon Pharma LLC is not responsible for the contents of the article. The article reflects the authors’ position, which may differ from that of Zambon Pharma LLC
About the Authors
E. I. Kondratyeva
Federal State Budgetary Scientific Institution “Research Center for Medical Genetics”
Russian Federation
Russian Federation
Elena I. Kondratyeva, Doctor of Medicine, Professor, Head of the Scientific and Clinical Department of Cystic Fibrosis, Head of Genetics Department of Respiratory System Diseases, Higher and Additional Professional Education Institute.
ul. Moskvorech’e 1, Moscow, 1115478, Russia; tel.: (495) 111-03-03
E. L. Amelina
Federal State Budgetary Scientific Institution “Research Center for Medical Genetics”; Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia
Russian Federation
Russian Federation
Elena L. Amelina, Candidate of Medicine, Head of the Laboratory of Cystic Fibrosis; Associate Professor, Department of Genetics of Respiratory System Diseases.
ul. Moskvorech’e 1, Moscow, 1115478, Russia; Orekhovyy bul’var 28, Moscow, 115682, Russia; tel.: (926) 205-03-91
M. Yu. Chernukha
Honorary Academician N.F.Gamalei Federal Research Center for Epidemiology and Microbiology, Healthcare Ministry of Russia
Russian Federation
Russian Federation
Marina Yu. Chernukha, Doctor of Medicine, Professor, Leading Researcher, Laboratory of Molecular Epidemiology of Hospital Infections.
ul. Gamalei 16, Moscow, 123098, Russia; tel.: (499) 193-55-94
V. D. Sherman
Federal State Budgetary Scientific Institution “Research Center for Medical Genetics”
Russian Federation
Russian Federation
Viktoriya D. Sherman, Candidate of Medicine, Leading Researcher, Scientific and Clinical Department of Cystic Fibrosis, Associate Professor, Genetics Department of Respiratory System Diseases, Institute of Higher and Additional Professional Education.
ul. Moskvorech’e 1, Moscow, 1115478, Russia; tel.: (916) 188-24-76
S. A. Krasovskiy
Federal State Budgetary Scientific Institution “Research Center for Medical Genetics”; Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia
Russian Federation
Russian Federation
Stanislav A. Krasovskiy, Candidate of Medicine, Senior Researcher, Cystic Fibrosis Laboratory; Senior Researcher, Scientific and Clinical Department of Cystic Fibrosis.
ul. Moskvorech’e 1, Moscow, 1115478, Russia; Orekhovyy bul’var 28, Moscow, 115682, Russia; tel.: (926) 273-76-34
N. Yu. Kashirskaya
Federal State Budgetary Scientific Institution “Research Center for Medical Genetics”
Russian Federation
Russian Federation
Nataliya Yu. Kashirskaya, Doctor of Medicine, Professor, Chief Researcher, Laboratory of Genetic Epidemiology.
ul. Moskvorech’e 1, Moscow, 1115478, Russia; tel.: (499) 320-60-90
O. I. Simonova
Federal Academic Centre of Children’s Health, Healthcare Ministry of Russia; I.M.Sechenov First Moscow State Medical University (Sechenov University), Healthcare Ministry of Russia
Russian Federation
Russian Federation
Ol’ga I. Simonova, Doctor of Medicine, Pediatrician, Head of the Pulmonary Department.
Lomonosovskiy pr. 2, build. 1, Moscow, 119991, Russia; ul. Trubetskaya 8, build. 2, Moscow, 119991, Russia; tel.: (910) 468-87-89
S. N. Avdeev
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; I.M.Sechenov First Moscow State Medical University (Sechenov University), Healthcare Ministry of Russia
Russian Federation
Russian Federation
Sergey N. Avdeev, Doctor of Medicine, Professor, Corresponding Member of Russian Academy of Sciences, Chief Pulmonologist, Healthcare Ministry of Russia, Head of the Pulmonology Department, N.V.Sklifosovsky Institute of Clinical Medicine; Leading Researcher.
Orekhovyy bul’var 28, Moscow, 115682, Russia; ul. Trubetskaya 8, build. 2, Moscow, 119991, Russia; tel.: (916) 614-58-25
L. S. Namazova-Baranova
Pediatrics and Child Health Research Institute of the “Central Clinical Hospital of the Russian Academy of Sciences”, Ministry of Education and Science of Russia
Russian Federation
Russian Federation
Leyla S. Namazova-Baranova, Doctor of Medicine, Professor, Academician of the Russian Academy of Sciences, Head; Chief Pediatric Specialist in Preventive Medicine, Healthcare Ministry of Russia; President of the Pediatricians Union of Russia.
Litovskiy bul’var 1A, Moscow, 117593, Russia; tel.: (499) 400-47-33
T. E. Gembitskaya
Academician I.P.Pavlov First Federal Saint-Petersburg State Medical University, Healthcare Ministry of Russia
Russian Federation
Russian Federation
Tat’yana E. Gembitskaya, Doctor of Medicine, Professor, Head of the Therapeutic Pulmonology Department, Research Institute of Pulmonology, Scientific and Clinical Research Center.
ul. L’va Tolstogo 6–8, Saint-Petersburg, 197089, Russia; tel.: (812) 338-66-25
S. I. Kutsev
Federal State Budgetary Scientific Institution “Research Center for Medical Genetics”
Russian Federation
Russian Federation
Sergey I. Kutsev, Doctor of Medicine, Professor, Corresponding Member of Russian Academy of Sciences, Chief supernumerary specialist in medical genetics, Healthcare Ministry of Russia, President of the Association of Medical Genetics, Director.
ul. Moskvorech’e 1, Moscow, 1115478, Russia; tel.: (499) 612-00-37
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For citations:
Kondratyeva E.I., Amelina E.L., Chernukha M.Yu., Sherman V.D., Krasovskiy S.A., Kashirskaya N.Yu., Simonova O.I., Avdeev S.N., Namazova-Baranova L.S., Gembitskaya T.E., Kutsev S.I. Review of clinical guidelines “Cystic fibrosis”, 2020. PULMONOLOGIYA. 2021;31(2):135-146. (In Russ.) https://doi.org/10.18093/0869-0189-2021-31-2-135-146
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