DRUG TREATMENT OF CYSTIC FIBROSIS PATIENTS IN RUSSIAN FEDERATION ACCORDING TO THE NATIONAL REGISTER, 2014
https://doi.org/10.18093/0869-0189-2016-26-5-539-555
Abstract
The aim of this study was to analyze drug therapy of patients with cystic fibrosis (CF) in Russia. Methods. Findings of the Russian National Register of CF patients, 2014, were used in this study. The 2014 Register included data for 2,131 patients (2,092 alive and 39 died) from 74 regions of Russia. The median age was 10.2 (15.2) years, 29.2% of patients were aged 18 years and older. We analyzed administration of bronchodilators, inhaled corticosteroids (ICS), systemic steroids, dornase alfa, hypertonic saline solution, macrolides, systemic and inhaled antibiotics, pancreatic enzymes, ursodeoxycholic acid, and fat-soluble vitamins. Results. Drug administration rates were as follows: bronchodilators, 65.9%; ICS, 21.7%; systemic steroids, 5.5%; macrolides, 32.4%; dornase-alfa, 92.8%; hypertonic saline solution, 45.9%; intravenous antibiotics, 62.3%; oral antibiotics, 73.3%; inhaled antibiotics, 41.3%; pancreatic enzymes, 93.3%; ursodeoxycholic acid, 91.4%; and fat-soluble vitamins, 88.3%. Conclusion. Compared to treatment of CF patients in the Western Europe, there is higher rate of administration of pancreatic enzymes, ursodeoxycholic acid, dornase alfa, hypertonic saline solution and intravenous antibiotics in Russia. Compared to treatment strategy in USA, there is higher rate of administration of pancreatic enzymes and lower rate of administration of bronchodilators, hypertonic saline solution and inhaled antibiotics in Russia.
About the Authors
S. A. Krasovskiy
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia: 32, 11th Parkovaya str., Moscow, 105077, Russia;
D.D.Pletnev City Clinical Hospital, Moscow Healthcare Department: 32, 11th Parkovaya str., Moscow, 105077, Russia;
Federal Medical Genetic Academic Center, Russian Academy of Science: 1, Moskvorech'e str., Moscow, 1115478, Russia;
Russian Federation
PhD, Senior Researcher at Laboratory of Cystic Fibrosis, Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; pneumologist at the 2nd Pulmonology Department of D.D.Pletnev City Clinical Hospital, Moscow Healthcare Department; Senior Researcher at Research and Clinical Division of Cystic Fibrosis, Federal Medical Genetic Academic Center, Russian Academy of Science
Russian Federation
PhD, Senior Researcher at Laboratory of Cystic Fibrosis, Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; pneumologist at the 2nd Pulmonology Department of D.D.Pletnev City Clinical Hospital, Moscow Healthcare Department; Senior Researcher at Research and Clinical Division of Cystic Fibrosis, Federal Medical Genetic Academic Center, Russian Academy of Science
E. L. Amelina
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia: 32, 11th Parkovaya str., Moscow, 105077, Russia
Russian Federation
PhD, Head of Laboratory of Cystic Fibrosis
Russian Federation
PhD, Head of Laboratory of Cystic Fibrosis
E. I. Kondrat'eva
Federal Medical Genetic Academic Center, Russian Academy of Science: 1, Moskvorech'e str., Moscow, 1115478, Russia
Russian Federation
MD, Professor, Principal Researcher at Research and Clinical Division of Cystic Fibrosis
Russian Federation
MD, Professor, Principal Researcher at Research and Clinical Division of Cystic Fibrosis
A. Yu. Voronkova
Federal Medical Genetic Academic Center, Russian Academy of Science: 1, Moskvorech'e str., Moscow, 1115478, Russia
Russian Federation
PhD, Senior Researcher at Research and Clinical Division of Cystic Fibrosis
Russian Federation
PhD, Senior Researcher at Research and Clinical Division of Cystic Fibrosis
A. V. Chernyak
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia: 32, 11th Parkovaya str., Moscow, 105077, Russia
Russian Federation
PhD, Head of Laboratory of Functional and Ultra-sound Investigations
Russian Federation
PhD, Head of Laboratory of Functional and Ultra-sound Investigations
Yu. V. Gorinova
Federal Academic Centre of Children's Health, Healthcare Ministry of Russia: 2, build.1, Lomonosovskiy av., Moscow, 119991, Russia
Russian Federation
PhD, Senior Researcher at Division of Pulmonology and Allergy
Russian Federation
PhD, Senior Researcher at Division of Pulmonology and Allergy
M. V. Usacheva
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia: 32, 11th Parkovaya str., Moscow, 105077, Russia
Russian Federation
Researcher at Laboratory of Cystic Fibrosis
Russian Federation
Researcher at Laboratory of Cystic Fibrosis
N. P. Revel'-Muroz
Chelyabinsk Regional Clinical Hospital: 70, Vorovskogo str., Chelyabinsk, 454076, Russia;
Russian Federation
PhD, Head of Department of Pulmonology
Russian Federation
PhD, Head of Department of Pulmonology
N. I. Romanenko
Novosibirsk City Pediatric Emergency Clinical Hospital: 3, Krasnyy av., Novosibirsk, 630007, Russia
Russian Federation
PhD, Head of Pediatric Department
Russian Federation
PhD, Head of Pediatric Department
M. G. Rybalkina
Orenburg State Medical Academy, Healthcare Ministry of Russia: 6, Sovetskaya str., Orenburg, 460000, Russia
Russian Federation
PhD, Assistant Lecturer at Department of Hospital Pediatrics
Russian Federation
PhD, Assistant Lecturer at Department of Hospital Pediatrics
T. I. Safonova
Omsk Regional Pediatric Clinical Hospital: 77, Kuybysheva str., Omsk, 644001, Russia
Russian Federation
Head of Pulmonology Department
Russian Federation
Head of Pulmonology Department
N. A. Satsuk
Nizhnevartovsk District Pediatric Clinical Hospital, Khanty Mansiysk – Yugrа autonomous district: 30, Severnaya str., Nizhnevartovsk, 628609, Russia
Russian Federation
Head of Pulmonology Department
Russian Federation
Head of Pulmonology Department
D. F. Sergienko
Astrakhan' State Medical Academy, Healthcare Ministry of Russia: 121, Bakinskaya str., Astrakhan', 414000, Russia
Russian Federation
MD, Professor at Department of General Pediatrics
Russian Federation
MD, Professor at Department of General Pediatrics
V. N. Seroklinov
Altay State Medical University, Healthcare Ministry of Russia: 75, Lenina av., Barnaul, 656060, Russia
Russian Federation
PhD, Associate Professor at Department of Pediatrics, Faculty of Postgraduate Physician Training
Russian Federation
PhD, Associate Professor at Department of Pediatrics, Faculty of Postgraduate Physician Training
T. V. Simanova
Udmurtia Republic Pediatric Clinical Hospital, Healthcare Ministry of the Udmurtia Republic: 79, Lenina str., Izhevsk, 426009, Udmurtia Republic
Russian Federation
PhD, Head of Pulmonology Department
Russian Federation
PhD, Head of Pulmonology Department
O. I. Simonova
Federal Academic Centre of Children's Health, Healthcare Ministry of Russia: 2, build.1, Lomonosovskiy av., Moscow, 119991, Russia
MD, Head of Department of Pulmonology and Allergology
MD, Head of Department of Pulmonology and Allergology
M. A. Skachkova
Novosibirsk City Pediatric Emergency Clinical Hospital: 3, Krasnyy av., Novosibirsk, 630007, Russia
MD, Professor, Head of Department of Hospital Pediatrics
MD, Professor, Head of Department of Hospital Pediatrics
V. V. Smirnova
N.V.Dmitrieva Ryazan’ Regional Pediatric Clinical Hospital: 1z, Internatsional'naya str., Ryazan’, 390039, Russia
PhD, Head of Pulmonology Department
PhD, Head of Pulmonology Department
I. I. Smirnova
Federal Research Institute of Medical Genetics, Siberian Department of Russian Academy of Medical Science: 3, Moskovskiy trakt, Tomsk, 634050, Russia
Competing Interests: Pediatrician
Competing Interests: Pediatrician
O. I. Starodubtseva
The First Republic Clinical Hospital, Healthcare Ministry of the Udmurtia Repiblic: 57, Votkinskoe av., Izhevsk, 426039, the Udmurtia Repiblic
PhD, Head of Pulmonology Department
PhD, Head of Pulmonology Department
T. V. Stashkevich
Bryansk Regional Pediatric Hospital: 100, Stanke Dimitrova av., Bryansk, 241033, Russia
Head of Pulmonology Department
Head of Pulmonology Department
T. A. Stepanenko
Saint-Petersburg City Multi-Profile Hospital No.2: 5, Uchebnyy side street, Saint-Petersburg, 194354, Russia
PhD, Head of Pulmonology Department
PhD, Head of Pulmonology Department
V. V. Chikunov
V.F.Voyno-Yasenetskiy Krasnoyarsk State Medical University, Healthcare Ministry of Russia: 1, Partizana Zheleznyaka str., Krasnoyarsk, 660022, Russia
PhD, Assistant Lecturer at Department of Pediatrics
PhD, Assistant Lecturer at Department of Pediatrics
L. A. Shabalova
Federal Medical Genetic Academic Center, Russian Academy of Science: 1, Moskvorech'e str., Moscow, 1115478, Russia
PhD, Senior Researcher at Research and Clinical Division of Cystic Fibrosis
PhD, Senior Researcher at Research and Clinical Division of Cystic Fibrosis
O. I. Golubtsova
Chuvash Republic Pediatric Clinical Hospital, Chuvash Healthcare Ministry: 27, Gladkova str., Cheboksary, 428020, the Republic of Chuvash
PhD, Head of Division of Pulmonology and Allergy
PhD, Head of Division of Pulmonology and Allergy
V. V. Shadrina
E.A.Vagner Perm State Medical Academy Healthcare Ministry of Russia: 22, Baumana str., Perm, 614066, Russia
PhD, Assistant Lecturer at Department of Hospital Pediatrics
PhD, Assistant Lecturer at Department of Hospital Pediatrics
O. G. Zonenko
N.I.Pirogov Russian State National Research Medical University, Healthcare Ministry of Russia: 1, Ostrovityanova str., Moscow, 117997, Russia
Assistant Lecturer
Assistant Lecturer
A. A. Shevlyakova
Omsk Regional Pediatric Clinical Hospital: 77, Kuybysheva str., Omsk, 644001, Russia
Pneumologist
Pneumologist
V. D. Sherman
Federal Medical Genetic Academic Center, Russian Academy of Science: 1, Moskvorech'e str., Moscow, 1115478, Russia
PhD, Senior Researcher at Research and Clinical Division of Cystic Fibrosis
PhD, Senior Researcher at Research and Clinical Division of Cystic Fibrosis
N. A. Karchevskaya
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia: 32, 11th Parkovaya str., Moscow, 105077, Russia
Researcher at Laboratory of Non-invasive Diagnostics
Researcher at Laboratory of Non-invasive Diagnostics
Zh. K. Naumenko
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia: 32, 11th Parkovaya str., Moscow, 105077, Russia
PhD, Senior Researcher at Laboratory of Functional and Ultra-sound Investigations
PhD, Senior Researcher at Laboratory of Functional and Ultra-sound Investigations
I. N. Butyugina
D.D.Pletnev City Clinical Hospital, Moscow Healthcare Department: 32, 11th Parkovaya str., Moscow, 105077, Russia
Pneumologist at the 2nd Pulmonology Department
Pneumologist at the 2nd Pulmonology Department
O. Yu. Gracheva
D.D.Pletnev City Clinical Hospital, Moscow Healthcare Department: 32, 11th Parkovaya str., Moscow, 105077, Russia
Head of the 2nd Pulmonology Department
Head of the 2nd Pulmonology Department
M. S. Kevorkova
N.I.Pirogov Russian State National Research Medical University, Healthcare Ministry of Russia: 1, Ostrovityanova str., Moscow, 117997, Russia
PhD student
PhD student
V. B. Yakovleva
V.D.Seredavin Samara State Regional Clinical Hospital: 159, Tashkentskaya str., Samara, 443095, Russia
Pneumologist at Pulmonology Department
Pneumologist at Pulmonology Department
N. Yu. Kashirskaya
Federal Medical Genetic Academic Center, Russian Academy of Science: 1, Moskvorech'e str., Moscow, 1115478, Russia
MD, Professor, Principal Researcher at Laboratory of Genetic Epidemiology
MD, Professor, Principal Researcher at Laboratory of Genetic Epidemiology
Review
For citations:
Krasovskiy S.A., Amelina E.L., Kondrat'eva E.I., Voronkova A.Yu., Chernyak A.V., Gorinova Yu.V., Usacheva M.V., Revel'-Muroz N.P., Romanenko N.I., Rybalkina M.G., Safonova T.I., Satsuk N.A., Sergienko D.F., Seroklinov V.N., Simanova T.V., Simonova O.I., Skachkova M.A., Smirnova V.V., Smirnova I.I., Starodubtseva O.I., Stashkevich T.V., Stepanenko T.A., Chikunov V.V., Shabalova L.A., Golubtsova O.I., Shadrina V.V., Zonenko O.G., Shevlyakova A.A., Sherman V.D., Karchevskaya N.A., Naumenko Zh.K., Butyugina I.N., Gracheva O.Yu., Kevorkova M.S., Yakovleva V.B., Kashirskaya N.Yu. DRUG TREATMENT OF CYSTIC FIBROSIS PATIENTS IN RUSSIAN FEDERATION ACCORDING TO THE NATIONAL REGISTER, 2014. PULMONOLOGIYA. 2016;26(5):539-555. (In Russ.) https://doi.org/10.18093/0869-0189-2016-26-5-539-555
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