scholarly journals Epilemiological characteristics of Diamond–Blackfen anemia in pediatric population of the Russian Federation

2019 ◽  
Vol 18 (3) ◽  
pp. 22-28
Author(s):  
V. M. Chernov ◽  
G. S. Ovsyannikova ◽  
M. B. Yudina ◽  
A. V. Rogov ◽  
N. E. Sokolova ◽  
...  

Diamond–Blackfen Anemia (DBA) is a rare, clinically and genetically heterogeneous disorder from the group of congenital syndromes of bone marrow failure. The purpose of this work is to identify the main medical-frequency characteristics of DBA (incidence, prevalence, mortality, cartographic analysis) in children in the Russian Federation during the observation period 2011–2016. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The Russian register of DBA patients, which had been developed and maintained since 2012 year by NMITS DGOI, was used to obtained epidemiological data and their analysis. At the end of 2018 year 141 patients from 137 families were included in the register. The average annual incidence rate of children with DBA for the Russian Federation was 0.63 ± 0.034, the average annual prevalence rate – 5.75 ± 0.87 per 100 thousand newborns born alive; mortality rate – 2.12%; the cartographic method of research showed that the largest number of patients was registered in the Central Federal District of the country, which is explained by the high population living in this district.

Author(s):  
V. M. Rozinov ◽  
D. A. Morozov ◽  
S. A. Rumyantsev ◽  
N. N. Vaganov ◽  
A. K. Fedorov ◽  
...  

Introduction. Russia needs to reform the stepwise model for delivering medical aid to children with surgical diseases and traumas mobilizing patients and those injured at specialized Interregional Centers (IRC). Purpose. To substantiate profiles and dislocation of IRC providing surgical aid to children in Russia. Material and methods. 103 specialists from 85 territorial entities of the Russian Federation presented their expert reviews concerning profiles and dislocation of IRC that provide surgical aid to children in accordance with the nomenclature of professions and administrative structure of the country. The expert reviews were analyzed using the methods of descriptive statistics to rate IRC profiles, their territorial dislocation, and focus on interaction of those surveyed with certain medical organizations. Consistency of expert opinions was estimated using Kendall’s coefficient of concordance. Results. Experts indicated 14 activity profiles of IRC providing surgical aid to children. According to rating results, more than a half of positive estimates belonged to five priority profiles including surgery of neonates, neurosurgery, oncology, thoracic surgery and combustiolgy. As far as IRC dislocation is concerned, experts mainly considered medical organizations within a federal district of territorial entities of the Russian Federation. Total number (287) of references (by priority profiles) to hospitals within territorial entities of Russia was 4 times more than the respective estimate (66) for federally governed healthcare providers. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology that served as a base for potential IRC (oncology) was the most in-demand (19 requests) federal structure. Conclusion. Community of professionals has come to a consolidated opinion according to which buildup of IRC providing care in neonatal surgery, neurosurgery, oncology, thoracic surgery, and combustiology at children’s hospitals in territorial entities of the Russian Federation and 13 federally governed clinical research centers is a significant factor of providing affordable and qualitative medical aid.


Author(s):  
S. A. Kogan ◽  
Ya. A. Erdomaeva ◽  
T. G. Serik ◽  
D. V. Birlyukova ◽  
G. I. Serik ◽  
...  

The purpose of the study was to create a certification of the service of pediatric hematology-oncology of the constituent entities of the Russian Federation (RF) using infographics and digitalization. Materials and methods. For the first time, infographic maps were used as a tool for certification, as a way of visualizing a large amount of data on the organization of pediatric hematology-oncology service in each subject of the RF. They allowed to show the specifics of the medical and organizational structure of the profile service in the subjects of the RF, their interaction with other subjects and federal centers. Processing of the cartographic image of the subject of the RF was carried out in the Adobe Illustrator graphic editor. A modifiable tool for creating infographic maps created in the MS Power Point program, with saving the results in PDF and PNG formats. Results. In the course of the study, a “Passport of Service” was prepared, containing infographic maps of 77 constituent entities of the RF. The maps in a graphic form provide information on resources available in the subject of the RF: a medical organization of the 3rd level with bed structure and the possibility of visualization, its personnel; the number of multicenter protocols and clinical guidelines used in the subject of the RF; logistics of patients, taking into account the territorial distance from the specialized beds, the presence in the region of the station/department of blood transfusion; the possibility of radiotherapy in the region; the presence of institutions where they provide palliative and rehabilitation assistance; the name of the federal and regional specialized centers, with whom the interaction is carried out, the well-established telemedicine consultation system with the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The database of contacts of the main freelance children’s specialists of hematologists and oncologists of the RF was also updated. Conclusion.Thus, data were collected for visual display and to create specific conditions for developing optimal patient routing schemes and forming proposals for optimizing the service. The obtained “Passport of the Children’s Hematology-Oncology Service of the Russian Federation” can be used in the work of hospitals, scientific centers and professional communities.


2018 ◽  
Vol 5 (3) ◽  
pp. 155-163 ◽  
Author(s):  
M. Yu. Rykov ◽  
I. A. Turabov

Background.Analysis of statistical data in the federal districts of the Russian Federation provides opportunities to assess the quality of medical care delivery in children with cancer and plan further development with consideration for the point elimination of existing defects. Objective. Our aim was to analyze the main indicators characterizing medical care for children with cancer in the North-West Federal District.Methods.The study analyzed operative reports for 2017 provided by the executive public health authorities of 11 subjects of the Russian Federation affiliated to the North-West Federal District.Results.The pediatric population was 2 537 133 children (0–17 years), the pediatric oncological bed capacity — 174 (0.7 per 10 000 aged 0–17 years), the annual berth average occupancy — 290.7 days. 6 (54.5%) subjects did not provide pediatric population with departments of pediatric oncology, 4 (36.4%) subjects did not have bed capacity. The number of practitioners providing medical care to children with cancer was 38, 27 among them (71%; 0.1 per 10 thousand 0–17 years) had a certificate of pediatric oncologist. 2 (18.2%) subjects did not have pediatric oncologists in the medical stuff. The incidence of malignant tumors in children aged 0–17 was 15.6 (per 100,000), the prevalence was 92.6 (per 100,000), the mortality rate was 2.6 (per 100,000), one-year mortality rate — 4.5% (18/397). The number of patients identified actively was 11 (2.8%). 160 (40.3%) primary patients were sent to Federal medical facilities, 6 (1.5%) — left the territory of the Russian Federation for the further treatment.Conclusion.The incidence and mortality rates are rather low which indicates the defects in patient detection and lack of reliable follow-up data. The percentage of patients referred for treatment to Federal medical facilities is not very high; however, to interpret this indicator according to patient routing, we have to analyze the medical history of all the patients. We can assert that health care delivery service for children with oncological diseases is at an acceptable standard level but needs to be improved. 


Author(s):  
O.V. Kolenko ◽  
◽  
M.V. Pshenichnov ◽  
E.L. Sorokin ◽  
M.G. Grekov ◽  
...  

Purpose. Evaluation of the first experience in organizing systematic telemedicine consultations/conciliums between the national medical research center and medical organizations in the territory of the Far Eastern Federal District (FEFD) of the Russian Federation. Material and methods. The analysis of organizational and technical difficulties in creating of stable system of planned and unscheduled telecommunication events between the Khabarovsk branch of the Fyodorov Eye Microsurgery Federal State Institution (Khabarovsk branch) and assigned to it medical organizations of the 3rd level, providing ophthalmological care for patients from 8 administrative territories of the FEFD. Results. Organizational and methodological department was created in the Khabarovsk branch, which clarified and eliminated the factors that impeded of telemedicine consultations/conciliums for ophthalmologists of medical organizations of the 3rd level; a set of organizational measures was taken to register these medical organizations in the Federal Register of Medical Organizations, in the Unified State Information System in the Field of Healthcare; the equipment necessary for communication was purchased and technically prepared; methodological assistance was provided in setting up equipment in the regions, including on-site visits; backup connections of several providers have been created; an algorithm for preliminary notification of regional ophthalmologists about the knowledge transmission has been worked out; an organizational algorithm for forming a request for telemedicine consultations has been tested. For 2019–2020 the Khabarovsk branch conducted 32 knowledge transmissions, 560 telemedicine consultations on patients living in the FEFD. Conclusion. The introduction into the daily clinical ophthalmologists practice in the FEFD of system of periodic telecommunication events between the Khabarovsk branch and assigned medical organizations of the 3rd level after the elimination of organizational and technical difficulties showed a high demand, allowing consultations on patient management in accordance with modern standards of ophthalmological care. Key words: telemedicine, teleophthalmology, Far Eastern Federal District, knowledge transmission, telemedicine consultations


2021 ◽  
Vol 22 (3) ◽  
pp. 82-89
Author(s):  
T. V. Razumova ◽  
◽  
T. V. Zuyevskaya ◽  
P. I. Pavlov ◽  
E. V. Sadrieva ◽  
...  

Purpose of the study: assessment of the level and structure of the incidence of the population to determine the main categories of diseases most common in the Khanty-Mansi Autonomous Okrug-Yugra, as well as making a territorial comparison of the incidence of the population. Materials and research methods: statistical materials from the annual collection “Health of the population of the Khanty-Mansi Autonomous Okrug-Yugra and the activities of medical organizations” and data of statistical reporting on the incidence of the population from the Medstat program were used, in particular, the primary morbidity of the population of the Khanty-Mansi Autonomous Okrug-Yugra in different age groups, the general incidence the population of different age groups of the Khanty-Mansi Autonomous Okrug-Yugra. The analysis used indicators such as primary morbidity, general morbidity, one-year mortality (died within the first year from the date of diagnosis) in malignant neoplasms. Results. The primary morbidity of the population in the Khanty-Mansi Autonomous Okrug-Yugra tends to grow in all age groups. The indicators of primary morbidity of the population of the Khanty-Mansi Autonomous Okrug-Yugra in 2018 are 17.1% higher than in the Russian Federation and 12.2% higher than in the Ural Federal District. In the structure of primary morbidity, the first place is taken by diseases of the respiratory system, the second place in the structure of the primary morbidity of people over working age is taken by diseases of the circulatory system, and the third place is by diseases of the genitourinary system. The overall incidence rate of the entire population is 1896 per 1000 population. In 2018, 41.2 cases of tuberculosis per 100 thousand of the population were detected for the first time in the Khanty-Mansi Autonomous Okrug-Yugra (in the Ural Federal District – 61.6, in the Russian Federation – 44.4 per 100 thousand of the population). This pathology tends to decrease and compared with 2017 decreased by 10.2% (the Russian Federation decreased by 8.1%, the Ural Federal District – 2.1%). The number of patients with newly diagnosed HIV infection in 2018 decreased by 1.5% compared to 2017 and amounted to 82.3 per 100 thousand population, which is higher than in the Russian Federation (58.5), but lower. than in the Ural Federal District (108.2) per 100 thousand population. Since 2012, there has been a tendency towards an increase in the incidence of malignant neoplasms, but the detection rate of malignant neoplasms at stages III-IV has not changed since 2015 and is 39.5%, which is higher than in the Russian Federation (38.5%). Conclusion: the primary morbidity in the Khanty-Mansi Autonomous Okrug-Yugra in 2018 is 17.1% higher than in the Russian Federation. In the first place are diseases of the respiratory system (48.4%, of which in children – 66.4, and in persons of older working age – 22.2%). In second place in children is infectious pathology, and in people of working age – diseases of the genitourinary system. The second place in morbidity in older people of working age is diseases of the circulatory system. The district has a high level of infectious and parasitic diseases. The number of patients with newly diagnosed tuberculosis is lower than in the Ural Federal District and the Russian Federation. The number of patients with HIV infection is higher than in the Russian Federation, but lower than in the Ural Federal District. There is a tendency towards an increase in the number of malignant neoplasms. The number of neoplasms is lower than in the Ural Federal District and the Russian Federation. In the Khanty-Mansi Autonomous Okrug-Yugra, there is a positive trend in the reduction of socially significant diseases, such as tuberculosis, HIV infection, STIs, alcoholism, drug addiction, injuries and an increase in overall morbidity, including malignant neoplasms, which indicates the availability of medical care, health literacy of the population and carrying out appropriate preventive work.


Author(s):  
K. I. Kirgizov ◽  
S. A. Kogan ◽  
Ya. A. Erdomaeva ◽  
G. M. Muftakhova ◽  
T. G. Shlyakhtina ◽  
...  

The basis for constructing a vertically integrated management system in the healthcare sector in the Russian Federation is the model of functioning of national centers. One of the key components in achieving success is the interaction of the national center with a leading national society. The aim of the article is to present the experience of joint work of the National Society of Pediatric Hematologists and Oncologists (NSPHO) with the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology in the field of development of pediatric oncology-hematology in the Russian Federation. In order to accomplish the task of building a system for organizing medical care for children in pediatric oncology-hematology, a comprehensive development program was implemented, which included collecting data on the structure of the service through questionnaires in the regions, obtaining expert opinion and the subsequent creation of a service passport using data visualization. In addition, the experience of creating professional standards and the concept of a unified approach in the field of training in the specialties “Pediatric Oncology” and “Hematology” using the technology of continuing medical education are reflected. The result of the work was the formation of a single plan for the organization of the service of pediatric oncology-hematology, developed by the NSPHO in conjunction with the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology with the support of the National Medical House and under the leadership of the Ministry of Health of Russia.


2020 ◽  
Vol 19 (3) ◽  
pp. 54-60 ◽  
Author(s):  
A. V. Poletaev ◽  
E. A. Seregina ◽  
D. V. Fedorova ◽  
P. A. Zharkov

The diagnosis of von Willebrand disease (vWD) in children remains a challenge in many regions of our country. This encouraged the Russian Hemophilia Society to create, in 2019, a special diagnostic programme offering remote diagnosis of vWD to patients in regions. Objectives: An interim evaluation of the effectiveness of the programme for remote diagnosis of vWD in children. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. Under this programme, if advised by a hematologist at the place of residence, a patient's peripheral blood was collected, frozen and sent to the Clinical Hemostasis Laboratory at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Over the period from 11.04.2019 to 07.08.2020, we received 72 frozen peripheral blood samples from children under the age of 18 sent from 21 regions of the Russian Federation. Von Willebrand factor (vWF) ristocetin cofactor activity, vWF antigen levels as well as coagulation factor VIII activity were measured. Out of the 72 samples, only one (1.4%) was inadequate. Reduced vWF activity (30–50%) was observed in 16.7% of cases, and another 16.7% of patients had vWF activity < 30%. VWF antigen levels were 30–50 % in 13.9 % of patients and < 30% in 9.7% of cases. Among the patients with vWF activity < 30%, 1 patient (8.3%) had type 1 vWD, 10 patients (83.3%) – type 2 vWD, 1 child (8.3%) – type 3 vWD. Six children (8.3%) demonstrated reduced factor VIII activity while maintaining normal vWF activity and antigen levels. The coagulation factor VIII activity/vWF antigen ratio was decreased in 8 children (11.1%). The vWD remote diagnosis programme has allowed us to detect abnormalities in 30 patients (41.7%), 16.7% of whom demonstrated laboratory features characteristic of vWD. Eighteen children (25%) require further, more comprehensive laboratory testing. This programme has great potential, especially in underpopulated regions where the development of local diagnostic capabilities may not be economically viable.


Author(s):  
Yuriy I. Zacharchenko ◽  
V. V Belichenko

The Main Bureau of Medical and Social Expertise in Krasnodar Region, Krasnodar, 350000, Russian Federation This article presents analysis of the dynamics of total disability resulting from traumas of all kinds in persons of all ages in the Russian Federation, the Southern Federal District and its subjects during the 6-year period (2009-2014). We revealed the following trends of disability. First of all, the total disability rate decreased in the Russian Federation, the Southern Federal District and its subjects, especially in 2013-2014. The rate of disability was high in middle-aged persons and low in retirement-age persons. The situation was critical and disability rate was high in the Republic of Kalmykia during the whole observation period. We observed low disability in the Astrakhan Region.


2021 ◽  
Vol 67 (1) ◽  
pp. 44-50
Author(s):  
Vakhtang Merabishvili

Year-by-year lethality – the lethality of patients with malignant tumors in each year of observation - an indicator that determines the risk of death of patients, depending on the localization of tumors. Lethality of patients in the first year of follow-up includes posthumously registered patients with malignant tumors, and is the inverse criterion to the indicator of one-year observed survival. In subsequent years, the calculation of year-by-year lethality is carried out without posthumously registered patients. It is important to note that the calculation of partial mortality rates is carried out only for the number of patients who died from malignant tumors. It is necessary to exclude patients who have dropped out of observation at each year of observation. Calculation of the year-by-year lethality indicator is possible only if there are at least 100 primary registered patients. In February 2019, we created the database of the Population cancer registry. The North-Western Federal district (NWFD)of the Russian Federation, which includes more than 1 million patients (1211891 patients with posthumously registered patients and 1071112 cases without posthumously registered patients). Only reliable data was selected for development, the number of which was almost 10% less. In 2020, the database of the Population cancer registry of the NWFD of the Russian Federation increased by 73054 cases. We used the updated database in this study. The observation period is from 1994 to 2020. The year-by-year lethality study was conducted in total for all ICD-10 categories for the C00-96 neoplasm class. DB analysis showed that in the NWFD of the Russian Federation, the proportion of posthumously registered patients decreased from 1995 to 2018 (both sexes) from 21.1 to 8.1%. For 1995 – 1999, 2000 – 2004, the year-by-year lethality was calculated for 10 years of observation, the period 2005-2009 covered the year-by-year lethality for 7 years of observation, 2005-2009 for 5 years. We have considered the specifics of year-by-year lethality separately for men and women, for people from 0-34, 35-59, 60 years and older. We pay special attention to year-by-year lethality for patients with localized (stage I and II of the disease) and widespread process (stage III and IV, including patients without the specified stage of the disease, which, as a rule, by the level of survival belong to stage III and IV of the disease).


2019 ◽  
Vol 18 (2) ◽  
pp. 5-14 ◽  
Author(s):  
M. Yu. Rykov

Background. Planning the organization of medical care for children diagnosed with cancer as well as assessing the quality of care is based on the statistical data analysis.The purpose of the study was to analyze the main parameters characterizing medical care for children with cancer in the central Federal district.Material and methods. The reports of the executive authorities in the sphere of health protection of 18 subjects of the Russian Federation included into the central Federal district were analyzed for 2017.Results. The number of children aged 0–17 years was 6 824 049, the number of pediatric oncology departments was 9, the number of hospital beds for children with cancer was 464, the number of bed-days per year was 319.3. the number of physicians providing pediatric cancer care was 91, of them 64 (70.3 %) had a certificate of a pediatric oncologist. in 11 subjects of the Russian Federation, there were no departments of pediatric oncology, and in 1of them, there were no hospital beds for children with cancer. the number of patients newly diagnosed with cancer was 821. the number of patients who died of cancer was 156, of them 66 were diagnosed with cancer in 2017. the cancer incidence rate was 12 per 100,000 children aged 017 years; the mortality rate was 2.3 per 100,000 children aged 017 years. the one-year mortality rate was 8 %. the mean time taken to establish the diagnosis and the time interval between diagnosis and initiation of treatment was unknown, since the reports did not contain precise information. twelve (1.5 %) patients left the territory of the Russian Federation for receiving treatment outside the Russian Federation.Conclusion. The information provided in most reports was not statistically reliable. the cancer incidence rates were significantly lower than those in countries with high statistical reliability. For reliable estimation of the number of inpatient beds for children with cancer and the percentage of children referred for the treatment to the federal medical centers, it is necessary to implement a unified database for pediatric oncology in the Russian Federation.


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