scholarly journals Analysis of the prevalence of rare (orphan) diseases («List 24») and the dynamics of detection of children's patients in the subjects of the Russian Federation

2020 ◽  
pp. 25-26
Author(s):  
И.А. Комаров ◽  
Е.Ю. Красильникова ◽  
О.Ю. Александрова ◽  
Р.А. Зинченко ◽  
С.И. Куцев
Keyword(s):  
Russian Federation ◽  
Orphan Diseases ◽  
Annual Increase ◽  
Federal Register ◽  
The Russian Federation ◽  
Low Prevalence ◽  
Timely Treatment

Проведен анализ распространенности редких (орфанных) заболеваний («Перечень 24») и динамики выявляемости пациентов детского возраста в 85 регионах РФ в период 2013-2019 гг. Показан ежегодный прирост пациентов в Федеральном регистре (2013 г. - 4962 больных, 2014 г. - 6761, 2015 г. - 7038, 2016 г. - 7625, 2017 г. - 8245, 2018 г. - 8639, 2019 г. - 9088). Динамика доли детей с заболеваниями из «Перечня 24» среди всех больных колебалась в небольших пределах (в среднем 50%). Большинством заболеваний (16 из 24) болеют в основном дети, но они встречаются также у взрослых. Распространенность заболеваний значительно варьировала между субъектами РФ. Улучшение информированности, а также ведение Федерального регистра способствуют эффективной выявляемости и своевременному назначению терапии в детском возрасте. Однако низкие значения распространенности заболеваний, проявляющихся до 1-го года жизни, говорят о сложностях их диагностики и недостаточной выявляемости. The analysis of the prevalence of rare (orphan) diseases («List of 24») and the dynamics of detection of children’s patients in 85 subjects of the Russian Federation in the period 2013-2019 showed an annual increase in patients in the Federal register (2013 - 4962 patients, 2014 - 6761, 2015 - 7038, 2016 - 7625, 2017 - 8245, 2018 - 8639, 2019 - 9088). The dynamics of the share of children with diseases from the “List of 24” among all patients fluctuated within small limits (on average 50%). Most of the diseases (16 out of 24) are mostly between children, but they also occur in adults. The prevalence of diseases varied significantly between the subjects of the Russian Federation. Improving awareness, as well as maintaining a Federal register promote effective detection and timely treatment in children. However, the low prevalence of diseases that occur before the 1st year of life indicates the complexity of their diagnosis and lack of detection.

scholarly journals Orphan diseases and associated problems

2021 ◽  
Vol 12 (2) ◽  
pp. 28-35
Author(s):  
V. A. Shashel ◽  
V. N. Firsova ◽  
M. M. Trubilina ◽  
L. A. Podporina ◽  
N. A. Firsov
Keyword(s):  
Medical Care ◽  
Russian Federation ◽  
Rare Diseases ◽  
Western Europe ◽  
Relevant Literature ◽  
The United States ◽  
Orphan Diseases ◽  
The Russian Federation ◽  
Low Prevalence ◽  
Drug Provision

Objective: to study the main aspects of assistance to children with orphan diseases in the Russian Federation. Materials and methods: the most relevant literature sources were studied that covered a concept of orphan diseases in various countries of the world and in the Russian Federation as well as the tactics and regulation of mechanisms for helping patients with rare diseases. Results: the study showed that not all countries have legislative regulations of assistance to orphan patients. The United States and Western Europe are the most advanced in this regard. They have clear criteria for determining orphan pathology and a number of measures are taken to improve the quality of medical care for patients with rare diseases. These activities are not only aimed at improving the health care system but also encourage pharmaceutical companies to develop and produce medicines as well as contribute to the research in this area. The clinical cases covered in the article provide an idea of rare diseases, the complexity of their diagnosis, the severity of the course, and the drugs that are necessary to help patients. Conclusion: orphan diseases have been the focus of attention by the health system and national legislation in the past decades. Their extremely low prevalence in the human population creates difficulties with the timely diagnosis, provision of qualified medical care, and drug provision.

Comparison and analysis of the fda approved orphane drugs registerand the vital and essential drugs list of the Russian Federation

2020 ◽  
pp. 4-16
Author(s):  
D.S. Yurochkin ◽  
◽  
A.A. Leshkevich ◽  
Z.M. Golant ◽  
I.A. NarkevichSaint ◽  
...  
Keyword(s):  
United States ◽  
Russian Federation ◽  
Rare Diseases ◽  
Orphan Drugs ◽  
The United States ◽  
Orphan Diseases ◽  
Essential Drugs ◽  
The Russian Federation ◽  
Comparison And Analysis ◽  
The Government

The article presents the results of a comparison of the Orphan Drugs Register approved for use in the United States and the 2020 Vital and Essential Drugs List approved on October 12, 2019 by Order of the Government of the Russian Federation No. 2406-r. The comparison identified 305 international non-proprietary names relating to the main and/or auxiliary therapy for rare diseases. The analysis of the market of drugs included in the Vital and Essential Drugs List, which can be used to treat rare (orphan) diseases in Russia was conducted.

Features of the organization of the provision of medicines for the treatment of orphan diseases in the Russian Federation

2020 ◽  
Vol 23 (1) ◽  
pp. 22
Author(s):  
R.U. Khabriev ◽  
A.V. Grechko ◽  
V.S. Malichenko ◽  
Yu.N. Kashevarova ◽  
N.S. Volkova
Keyword(s):  
Russian Federation ◽  
Orphan Diseases ◽  
The Russian Federation

Study of the availability of gene therapeutic drugs in the Russian Federation

2021 ◽  
pp. 69-75
Author(s):  
Konstantin A. Koshechkin ◽  
Philip A. Romanov ◽  
Alexander A. Mokhov ◽  
Alexander L. Khokhlov
Keyword(s):  
Gene Therapy ◽  
Russian Federation ◽  
World Literature ◽  
World Market ◽  
European Market ◽  
Orphan Diseases ◽  
Therapeutic Drugs ◽  
The World ◽  
The Usa ◽  
The Russian Federation

The authors studied the availability of gene therapy drugs in the Russian Federation on the basis of information on the permission of the medical use of drugs of this group in the world. Literature data and information about medicines approved by the FDA, EMA and the Ministry of Health of Russia were used. In general, the FDA registered only 13 drugs (46%) of the total approved for medical use in the world, 2 of them have already been withdrawn from the market, and 2 additional clinical trials are underway. In Europe, the EMA has approved 16 drugs for medical use (57%), with 4 of them already withdrawn. Most of the drugs were first approved by the FDA, and then, on average, a year later, were approved in the European market. A total of 4 drugs were approved in the European market and were not approved by the FDA at the time the data was requested. And only 1 drug, approved in the USA, is not registered in Europe. In the Russian Federation, two medicines are allowed, Neovasculgen (2011) and Spinraza (2019). This is only 7% of the total number of gene therapy drugs on the world market. Most of the drugs are intended for the treatment of orphan diseases and are cost expensive. This can explain the unevenness of their distribution across regions.

FRCEC information resources in priority vectors of science and technology

2021 ◽  
Author(s):  
Nikolay Mironov ◽  
◽  
Vladislava Dolgova ◽  
Keyword(s):  
Higher Education ◽  
Russian Federation ◽  
Science And Technology ◽  
Information Resources ◽  
Information Support ◽  
Research Centre ◽  
Federal Register ◽  
Federal Research ◽  
Science And Education ◽  
The Russian Federation

Federal Research Centre for Projects Evaluation and Consulting Service (FRCEC) is one of the leading Russian institutes providing expertise, research, methodological, technological, organizational and information support of sci-tech and innovative activities in the country. In years, the Institute, now subordinate to the Ministry of Science and Higher Education of the Russian Federation, has developed the mechanisms of expertise and acquiring data on innovative achievements, their control and implementation. The Federal Register of Experts (FRE) has been continuously updated to include accredited authoritative scientists and professionals in priority sci-tech spheres. The FRE structure and functionality are described in detail. Beside FRE, several FRCEC information resources are also discussed in brief: information support of RF Presidential grants; system for controlling and monitoring minor innovative enterprises in science and education, information catalog of current innovative infrastructure of sci-tech activities in RF constituents, etc.

PREVALENCE AND DIAGNOSIS OF RARE (ORPHAN) DISEASES IN PEDIATRIC POPULATION OF THE RUSSIAN FEDERATION

2020 ◽  
Vol 99 (4) ◽  
pp. 229-237
Author(s):  
T.P. Vasilieva ◽  
◽  
R.A. Zinchenko ◽  
I.A. Komarov ◽  
E.Yu. Krasilnikova ◽  
...  
Keyword(s):  
Russian Federation ◽  
Pediatric Population ◽  
Orphan Diseases ◽  
The Russian Federation

scholarly journals Payment of Fines for Administrative Offenses to the Budget in the System of the Ministry of Internal Affairs of Russia: Problems and Possible Solutions

REGIONOLOGY ◽  
2018 ◽  
Vol 26 (3) ◽  
pp. 606-623
Author(s):  
Svetlana S. Artemyeva ◽  
Vladimir V. Piskunov
Keyword(s):  
Russian Federation ◽  
Scientific Research ◽  
Comprehensive Approach ◽  
Annual Increase ◽  
Constituent Entity ◽  
Construction Methods ◽  
Dialectical Approach ◽  
Economic Method ◽  
The Russian Federation ◽  
Internal Affairs

Introduction. The annual increase in the number of identified administrative offenses requires more effective measures to administer payments. Improvement of the pro- cess of administering fines is relevant both for the development of internal affairs agencies and for the country’s economy as a whole. The purpose of the paper is to identify the problems arising when administering fines for administrative offenses and to suggest ways to solve them. Materials and Methods. A comprehensive approach to the problem under discussion was used together with the dialectical approach to the research results. The statistical and economic method, as well as the abstract-logical, monographic, calculation and construction methods of scientific research were also employed. Results. It has been revealed that the administration of fines for administrative of- fenses by the subdivisions of the Ministry of Internal Affairs of the Russian Federation following “their own” various methods has not been effective. Indicators have been determined that made it possible to improve the situation taking into account the passage of payments to budget accounts of the country’s budget system. Problems with receipt of payments to the budget of a constituent entity of the Russian Federation have been identified. Measures have been proposed to develop the mechanism for execution of payments of administrative fines imposed by the internal affairs agencies and to strengthen control over the reception of payments for administrative offenses. Discussion and Conclusions. The annual increase in the number of administrative offenses requires more effective measures to administer payments as this greatly affects the level of revenues in all the budgets in the country. The measures proposed by the authors to centralize the reception of payments and strengthen control over their receipt will help to avoid numerous complaints of people and organizations concerning recording the facts of payment of fines, as well as to reduce the burden on administrators of budget revenues and reduce to minimum unidentifiable payments.

scholarly journals Features of the legal regulation of the use of biometric personal data

2021 ◽  
Vol 7 (4) ◽  
pp. 27-32
Author(s):  
Daria A. Petrova ◽  
Nikita R. Martianov
Keyword(s):  
Russian Federation ◽  
Personal Data ◽  
Legal Regulation ◽  
The State ◽  
Legal Basis ◽  
Biometric System ◽  
Federal Law ◽  
Federal Register ◽  
The Russian Federation ◽  
The Creation

In this paper, the authors highlight the legal basis for the use of biometric personal data, and also pay attention to the main initiative of the state in this area: the creation of a unified biometric system. The study is particularly relevant in connection with the release of the new Federal law No. 168-FZ on June 8, 2020: On the unified Federal information register containing information about the population of the Russian Federation. In particular, an acute problem is the ability to integrate information from the unified biometric system and the Federal register that is being created.

Sign in / Sign up

Export Citation Format

Share Document