Clinical, molecular, and microbiological characteristics of cystic fibrosis patients at Moscow region and Belarus’ Republic
https://doi.org/10.18093/0869-0189-2018-28-3-296-306
Abstract
The aim of this study was a comparative analysis of course and treatment of cystic fibrosis (CF) in patients living at Moscow Region or at Republic of Belarus’. Methods. This was a comparative analysis of CF patients living at Moscow region (n = 197) or at Republic of Belarus’ (n = 110). The following clinical data were evaluated: sweat chloride test results if available, sputum microflora, nutritional status, complications of CF, and the current treatment. Results. The patient samples did not differ in gender, age, and lung function and had similar rates of F508del and CFTRdele2,3 mutations and Pseudomonas aeruginosa infection. Similar methods were used in both groups to confirm the diagnosis. The groups differed significantly in prevalence of Staphylococcus aureus, Burkholderia cepacia complex, and non-tuberculosis mycobacteria infections. Adult patients living at Moscow Region have lower forced expiratory volume for 1 sec compared to those living at Republic of Belarus’. CF patients younger 18 years of age who lived at Moscow Region had higher body mass index that those living at Republic of Belarus’. CF patients living at Republic of Belarus’ had hepatic cirrhosis and nasal polyps more often that those living at Moscow Region. Conclusion. CF patients living at Moscow Region and at Republic of Belarus’ had similar health status. Children and adolescents from those regions did not differ in key parameters predicting life expectancy and quality of life in CF. The groups differed in the prevalence of hepatic cirrhosis and nasal polyps. Modern ambulatory management using novel inhaled mucolytic and antibacterial agents, and regular follow-up every 3 months allow maintaining the lung functional status and P. aeruginosa infection rate close to that of the patients managed with regular in-hospital intravenous antibacterial and steroid therapy.
About the Authors
E. I. Kondrat’yeva
Federal Medical Genetic Academic Center, Russian Academy of Science;
Moscow Regional State Referral Center for Children
Russian Federation
Russian Federation
Elena I. Kondrat'eva, Doctor of Medicine, Professor, Head of Research and Clinical Division of Cystic Fibrosis, Federal Medical Genetic Academic Center, Head of Department of Cystic Fibrosis, Moscow Regional State Referral Center for Children
ul. Moskvorech'e 1, Moscow, 1115478, ul. Kominterna 24A, build.1, Mytishchi of Moscow Region, 1141007
A. Yu. Voronkova
Federal Medical Genetic Academic Center, Russian Academy of Science;
Moscow Regional State Referral Center for Children
Russian Federation
Russian Federation
Anna Yu. Voronkova, Candidate of Medicine, Senior Researcher, Research and Clinical Division of Cystic Fibrosis, Federal Medical Genetic Academic Center; pediatrician at Department of Cystic Fibrosis, Moscow Regional State Referral Center for Children
ul. Moskvorech'e 1, Moscow, 1115478, ul. Kominterna 24A, build.1, Mytishchi of Moscow Region, 1141007
V. I. Bobrovnichiy
Belarus’ State Medical University
Belarus
Belarus
Vladimir I. Bobrovnichiy, Candidate of Medicine, Associate Professor, Department of Pediatric Diseases No.2
pr. Dzerzhinskogo 83, Minsk, 220116
V. D. Sherman
Federal Medical Genetic Academic Center, Russian Academy of Science;
Moscow Regional State Referral Center for Children
Russian Federation
Russian Federation
Viktoriya D. Sherman, Candidate of Medicine, Senior Researcher, Research and Clinical Division of Cystic Fibrosis, Federal Medical Genetic Academic Center; pediatrician at Moscow Regional State Referral Center for Children
ul. Moskvorech'e 1, Moscow, 1115478, ul. Kominterna 24A, build.1, Mytishchi of Moscow Region, 1141007
E. K. Zhekayte
Federal Medical Genetic Academic Center, Russian Academy of Science;
Moscow Regional State Referral Center for Children
Russian Federation
Russian Federation
Elena K. Zhekayte, Researcher, Research and Clinical Division of Cystic Fibrosis, Federal Medical Genetic Academic Center; pediatrician at Department of Cystic Fibrosis, Moscow Regional State Referral Center for Children
ul. Moskvorech'e 1, Moscow, 1115478, ul. Kominterna 24A, build.1, Mytishchi of Moscow Region, 1141007
V. S. Nikonova
Federal Medical Genetic Academic Center, Russian Academy of Science
Russian Federation
Russian Federation
Viktoriya S. Nikonova, Candidate of Medicine, Senior Researcher, Research and Clinical Division of Cystic Fibrosis
ul. Moskvorech'e 1, Moscow, 1115478
O. V. Kras’ko
State Research United Institute of Informatics, National Academy of Sciences of Belarus’
Belarus
Belarus
Ol’ga V. Kras’ko, Candidate of Engineering, Leading Researcher, Laboratory of Bioinformatics; Assistant Professor
ul. Surganova 6, Minsk, 220012
S. A. Krasovskiy
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia
Russian Federation
Russian Federation
Stanislav A. Krasovskiy, Candidate of Medicine, Senior Researcher, Laboratory of Cystic Fibrosis
Orekhovyy bul'var 28, Moscow,115682
N. V. Petrova
Federal Medical Genetic Academic Center, Russian Academy of Science
Russian Federation
Russian Federation
Nika V. Petrova, Doctor of Medicine, Professor, Chief Researcher, Laboratory of Genetic Epidemiology
ul. Moskvorech'e 1, Moscow, 1115478
N. N. Chakova
State Research Institute of Genetics and Cytology, National Academy of Sciences of Belarus’
Belarus
Belarus
Natal’ya N. Chakova, Candidate of Biology, Leading Researcher, Laboratory of Modelling of Genetic Processes
ul. Akademicheskaya 27, Minsk, 220072
O. G. Novoselova
Federal Medical Genetic Academic Center, Russian Academy of Science;
State Research Institute of Genetics and Cytology, National Academy of Sciences of Belarus’
Russian Federation
Russian Federation
Ol’ga G. Novoselova, Postgraduate student, Federal Medical Genetic Academic Center; a geneticist, Head of Medical Genetic Center, Clinical and Diagnostic Center, N.F.Filatov Moscow State Pediatric Teaching Hospital No.13, Moscow Healthcare Department
ul. Moskvorech'e 1, Moscow, 1115478, ul. Sadovaya-Kudrinskaya 15, Moscow, 123001
R. M. Budzinskiy
Federal Medical Genetic Academic Center, Russian Academy of Science;
Moscow Regional State Referral Center for Children
Russian Federation
Russian Federation
Roman M. Budzinskiy, Researcher, Research and Clinical Division of Cystic Fibrosis, Federal Medical Genetic Academic Center; pediatrician at Department of Cystic Fibrosis, Moscow Regional State Referral Center for Children
ul. Moskvorech'e 1, Moscow, 1115478, ul. Kominterna 24A, build.1, Mytishchi of Moscow Region, 1141007
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Review
For citations:
Kondrat’yeva E.I., Voronkova A.Yu., Bobrovnichiy V.I., Sherman V.D., Zhekayte E.K., Nikonova V.S., Kras’ko O.V., Krasovskiy S.A., Petrova N.V., Chakova N.N., Novoselova O.G., Budzinskiy R.M. Clinical, molecular, and microbiological characteristics of cystic fibrosis patients at Moscow region and Belarus’ Republic. PULMONOLOGIYA. 2018;28(3):296-306. (In Russ.) https://doi.org/10.18093/0869-0189-2018-28-3-296-306
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