scholarly journals The development of pediatric oncology-hematology in the Russian Federation: the experience of collaboration between the National Society of Pediatric Hematologists and Oncologists and the National Medical Research Center

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.

2021 ◽  
Vol 1 (3) ◽  
pp. 132-133
Author(s):  
Kh. A. Tengizov ◽  
S. V. Shahidzhanova ◽  
M. A. Saidova

This paper describes the experience of teaching transthoracic echocardiography (TTE) skills on simulation equipment, accumulated over 2 years of training of residents in the direction of "Cardiology" at the Federal State Budgetary Institution "National Medical Research Center of Cardiology" of the Ministry of Health of the Russian Federation (Cardiocenter).


Author(s):  
N. A. Es’kin ◽  
T. M. Andreeva

N.N. Priorov National Medical Research Center of Traumatology and Orthopaedics, Moscow, Russia Injuries and musculoskeletal disorders are the most widely spread diseases. It is shown that the majority of injured patients and patients with orthopedic pathology require an outpatient care. The state of specialized outpatient care is analyzed and the ways of its perfection are proposed. 


2021 ◽  
Vol 100 (3) ◽  
pp. 200-207
Author(s):  
A.V. Pshonkin ◽  
◽  
E.V. Polevichenko ◽  
A.G. Andruzskaya ◽  
N.V. Zhukov ◽  
...  

Objective of the study: to analyze children with various diagnoses of malignant neoplasms (MNs), who underwent treatment at the National Medical Research Center of Pediatric Hematology, Oncology, Immunology, recognized incurable due to the characteristics of the main disease, as well as determining the optimum approach in transferring the patient to the patronage of the palliative service in the place of residence. Materials and methods of research: the analysis included patients who were treated at the Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology from November 2018 to November 2020, – 116 patients aged 0 up to 18 years, who, in the course of their treatment, have been recognized incurable and discharged to the place of residence under the supervision of children's palliative services or left in the center for the severity of the condition. Results: the number of incurable patients at the National Medical Research Center of Pediatric Hematology, Oncology, Immunology for the period of the study was 116 (2,6%) out of 4,444 hospitalized. The majority – 99 (85%) of 116 patients – were discharged to their homes for palliative care. However, the algorithm developed at the center, which provides for the initial selection of the optimal palliative care regime in the hospital for short-term treatment, the assessment of risks of transport and its type (with or without medical personnel), made it possible to ensure safe transportation of all patients, as well as full control of symptoms during transportation. and at the first stages of the patient's stay at the place of residence. Subsequent palliative treatment of patients at the place of residence was also corrected, if necessary, with the participation of specialists of the center, either in person or by correspondence, which made it possible to provide adequate palliative therapy to all patients until the end of the need. Conclusion: early integration of primary palliative care into the activities of the Federal center providing specialized pediatric oncological care may be one of the successful models of interaction between pediatric oncology services and palliative care for children in the Russian Federation.


2020 ◽  
Vol 19 (4) ◽  
pp. 20-31
Author(s):  
E. A. Litvin ◽  
D. T. Utalieva ◽  
D. Yu. Kachanov ◽  
A. V. Pshonkin ◽  
M. Ya. Yadgarov ◽  
...  

13-cis-Retinoic acid is a differentiation agent for neuroblastoma cells and is a part of post-consolidation therapy for high-risk patients. The effectiveness of this therapeutic approach is currently under study. 26 patients with high-risk neuroblastoma treated at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology were included in the study of 13-cis-Retinoic acid pharmacokinetics by high-performance liquid chromatography assay with ultraviolet detector depending on the method of administration of drug (swallowed capsules or opened capsules before administration). This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The current study showed that the therapeutic concentration of > 2 μM when taking 13-cis-Retinoic acid at a dose of 160 mg/m2/day was achieved in two groups, regardless of the method of drug administration. However, plasma concentrations of 13-cis-Retinoic acid at 4 hours after administration on the 14th day of therapy were higher in the group of patients who swallowed the capsules (4.1 ± 1.8 μM), compared to those who could not do it (1.9 ± 1.5 μM) (p = 0.022). The introduction into the clinical practice of therapeutic drug monitoring of 13-cis-retinoic acid in high-risk neuroblastoma patients with an assessment of peak concentration and dose adjustment of the following courses may be an important point in the attempt to optimize postconsolidation therapy and improve prognosis.


2021 ◽  
Vol 20 (2) ◽  
pp. 84-96
Author(s):  
E. A. Cherniak ◽  
N. E. Sokolova ◽  
K. V. Semiglazova ◽  
I. N. Lavrentyeva ◽  
E. K. Donush ◽  
...  

The article presents retrospective data analysis of a cohort of patients with PKD (n = 41 patients, aged 4 months – 26,5 years, median of age – 5 years 1 month) who were examined at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology for unspecifid hereditary hemolytic anemia during the period 2013–2020. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. In all patients, the diagnosis was confimed by Next Generation sequencing (NGS). The homozygous mutations in the PKLR gene were found in 10 patients (24.39%), compound heterozygous mutations in 31 patients (75.61%), 77.78% of them were missense mutations. Gender distribution (male:female) was 1:1.73. At least once transfusion of erythrocyte suspension was required to 40 (97.56%) patients. The minimum age at the time of the debut of transfusion dependence was the fist day of life, the maximum was 4 years. Exchange blood transfusion was performed in 13 children, severe normocytic hyperregenerative anemia with transfusion of red blood cells in the fist days of life was noted in 12 children, at the 1st month of life – in 9 children, at the 2nd month of life – in 8 children, at the 3rd month – in 6 children, at the 5th month – in 2 children, at the 1st year – in 1 child, and 2 children underwent single transfusions on the background of infectious episodes at 3 and 4 years respectively. Splenectomy due to high transfusion dependence was performed in 10 patients: transfusion independence was achieved in 5 patients, in 5 – an increase in the interval between blood transfusions. Median of surgical intervention (9 patients): 7 years 4 months, minimum age – 1 year 4 months, maximum – 14 years 4 months. In total, 36 genotypes were described in 41 patients, among them were: c.1529G>A in 3 patients, c.1137_1139del / c.1456C>T – in 2 patients, c.1079G>A/c.1529G>A in 2 patients, c.1130T>C/c.1456C>T in 2 patients, other genotypes occurred once. Two mutations were the most frequent: c.1456C>T (16.67%) and c.1529G>A (16.67%). 19 (46,34%) of patients had previously not described mutations.


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.


2019 ◽  
Vol 18 (4) ◽  
pp. 19-28
Author(s):  
T. V. Shamanskaya ◽  
D. Y. Kachanov ◽  
A. V. Dumacheva ◽  
M. V. Teleshova ◽  
D. V. Shevtcov ◽  
...  

High-risk neuroblastoma (NB) is characterized by unsatisfactory treatment results and low probability of long-term survival despite the multimodal therapeutic approach (chemotherapy, surgical treatment, radiation therapy, autologous hematopoietic stem cell transplantation, etc.). One of the prognostic factors in this cohort of patients is the response to induction therapy. The article presents the experience of the intensification of induction therapy in 12 patients with high-risk NB with a poor response (mixed response, stable disease) to standard induction therapy who received treatment at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, assessing its impact on the prognosis of the disease. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Patients received an additional two courses of chemotherapy with the inclusion of a type I topoisomerase inhibitor topotecan (TCE – topotecan, cyclophosphamide, etoposide). This regimen of intensification of therapy has demonstrated its feasibility. The main grade 3–4 toxicity was hematologic. An improvement in response was achieved in 5/12 (41.6%) patients. However, long-term results of therapy remained unsatisfactory. The 3-year EFS was 16.7% (95% CI 0.0–37.8), the 3-year OS was 50.0% (95% CI 21.7–78.3). Thus, the intensification of therapy in patients with high-risk NB with a poor response to standard induction therapy did not improve treatment outcomes.


Author(s):  
D. V. Shevtsov ◽  
T. V. Shamanskaya ◽  
D. Yu. Kachanov ◽  
N. S. Grachev ◽  
K. I. Kirgizov ◽  
...  

Introduction.Neuroblastoma (NB) is the most common extracranial solid tumor in children. As a rule, NB is localized in the adrenal gland, retroperitoneal space and posterior mediastinum. The head and neck area belongs to the rare localization of NB, which accounts for 2.6 % of cases, and is most common in children aged 0–3 years. Localization of NB in the neck in most cases has a favorable prognosis.Materials and methods.For the period from September 2013 to September 2017 (48 months) in the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology received treatment for 8 patients with NB in the neck. Examination, assessment of the prevalence of the process and stratification into risk groups in all patients were carried out according to the recommendations of the protocol of the German group for the treatment of NB NB-2004. For the purpose of histological verification of the diagnosis and detection of unfavorable molecular genetic markers, patients underwent surgical intervention, performed risk-adapted therapy according to the NB-2004 protocol.Results.The median age of diagnosis was 8.7 (1.2–34.1) months. In our cohort of patients in 87.5 % of cases, the diagnosis was made in the first year of life. In most cases, there was not only the identification of tumor masses, but also other symptoms of the disease. In 3 (37.5 %) patients the 2nd stage was established, in 1 (12.5 %) patient – the 3rd stage, in 3 (37.5 %) patients – the 4th stage and in 1 (12.5 %) patient – 4S stage of the disease. When stratifying patients into risk groups, in the observation group and the high-risk group was stratified by 3 (37.5 %) children and 2 (25 %) patients were classified as high-risk group. 3 (37.5 %) patients showed unfavorable cytogenetic abnormalities. When evaluating the response to therapy in most patients, a complete and very good partial response was stated. Overall (OS) and event-free (EFS) survival rates were 75 ± 15 % and 50 ± 17 %, respectively. The median of observation is 43 (26–61) months.Discussion.NB with the localization of the primary tumor in the head and neck area is a favorable form in terms of the stage of the disease and the risk group, however, it should be noted that in our patient cohort half of the subjects showed the development of certain adverse events, which was also reflected in the OS and EFS. Moreover, this localization dictates its risks from the point of view of the surgical stage of treatment. The main danger is complications after surgical treatment associated with the anatomical proximity of the central arteriovenous trunks, cranial nerves, and their involvement in the tumor process. In the case of the development of life threatening conditions (LTC), it is possible to use low-intensity chemotherapy courses.Conclusion.Experience Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology shows the need for timely diagnosis and the start of treatment of NB with localization in the neck. The choice of management tactics in favor of carrying out only surgical treatment is possible in patients of the observation group without the development of LTC. Not always the localization of NB in the neck region correlates with a favorable prognosis.Conflict of interest. The authors declare no conflict of interest.Funding. The study was performed without external funding.


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