TARGET THERAPY FOR PATIENTS WITH NEUROFIBROMATOSIS (LITERATURE REVIEW)

Актуальность представленного обзора обусловлена высокой частотой нейрофиброматоза 1 типа (NF1) и нейрофиброматоз 2 типа (NF2) в популяции и частой ассоциацией с развитием злокачественных новообразований. Целью подготовки обзора является анализ данных литературы, которые описывают результаты применения таргетных препаратов лечения новообразований у пациентов с нейрофиброматозом Результатами литературного поиска стало обобщение современных подходов к проведению таргетной терапии нейрофброматоза как NF1, так и NF2 типов. Представлены данные о фармакогенетике и принципах этиопатогенетической терапии с использованием следующих препаратов таргетной терапии: бевацизумаб, эрлотиниб, иматиниб, сорафениб., сиролимус. Сводная таблица с результатами обзора содержит сведения из 39 литературных источников. По мнению большинства авторов, инновационная таргетная терапия нейрофиброматоза может оказаться эффективной у многих пациентов с распространёнными формами заболевания. The relevance of the presented review is due to the high incidence of type 1 neurofibromatosis (NF1) and type 2 neurofibromatosis (NF2) in the population and frequent association with the development of malignant neoplasms. The purpose of the review is to analyze literature data that describe the results of the use of targeted drugs for the treatment of neoplasms in patients with neurofibromatosis. The results of the literary search became a generalization of modern approaches to targeted therapy of neurofromatosis both NF1 and NF2 types. The results of the literary search became a generalization of modern approaches to targeted therapy of neurofromatosis both NF1 and NF2 types. Data on pharmacogenetics and the principles of etiopathogenetic therapy with the use of the following targeted therapy are presented: bevacizumab, erlotinib, imatinib, sorafenib, sirolimus. The summary table with the results of the survey contains information from 39 literature sources. According to the majority of authors, innovative target therapy of neurofibromatosis can be effective in many patients with advanced forms of the disease

Wrongfulness of the act and current issues of legal regulation

It is important that in determining the delictual capacity of an individual, his guilt and the nature of mental attitude to the deed, which can be expressed in the form of intent and negligence, should be taken into account. This requires the establishment of three signs, including awareness by a person of unlawfulness of committed actions, anticipation by a person of the onset in socially harmful (socially dangerous) consequences and a person's attitude to the consequences that have occurred. It should be taken into account that during the reform of the criminal law, close attention should be focused on the presence of a person's motivation for unlawful behavior and the factors that determine it. Among such factors it is proposed to include biological features of personality, including sex, age, state of physical and mental health, state of nervous system, reaction to external stimuli, the presence of pathological deviations, character traits, temperament, level of emotional perception, intellectual and mental abilities

On the Prospects of Using Metallic Glasses for In-vessel Mirrors for Plasma Diagnostics in ITER

This chapter reviews main results obtained on mirror-like samples made of several grades of bulk metallic glasses (BMG). Experiments were carried out under simulated conditions typical for the operation of plasma facing in-vessel mirrors of optical plasma diagnostics in fusion reactor ITER. Bombardment with D0 and T0 atoms radiated from burning plasma was predicted to be the main reason for the degradation of optical properties of such mirrors. Therefore, to simulate the behavior of mirrors in ITER, mirror-like samples were subjected to bombardment by ions of deuterium plasma with fixed or wide energy distribution. The effects of ion bombardment on optical properties, development of roughness, uptake of deuterium, appearance of blisters, and manifestation of some chemical processes are presented and discussed

Analysis of the Russian Federation constituent regions’ sustainable development goals achievement

The article analyzes the Russian Federation constituent regions’ sustainable development goals (SDGs) achievement. Identified are the determinant Russian regions, the driving force and the reason for their coherence in achieving sustainable development goals. The study is based on a systematic approach using the method of statistical, neural network and cluster analysis. Results. The study has revealed a contradiction expressed in priority of “SDG 7. Fixed capital investments by areas of investment in terms of “Production of electricity from renewable energy sources, including generated by solar, wind, geothermal power plants, as well as activities to ensure their performance”” line of business and underperformance in terms of “SDG 16. Russia’s position in the rating of the World Bank’s Doing Business project in terms of the “Getting credit” indicator. The work shows priority in relation to “SDG 3. Life expectancy at birth”, which is implemented through national projects. Analysis of the large Russian clusters regional importance identified the Moscow region by “SDG 1. Proportion of the population living below the national poverty line” and “SDG 3. Life expectancy at birth”. The analysis of interregional ties revealed determinant regions, the existence of which is supported by the dynamics of the developing Russian market, which is far behind countries with developing economies

Digital spectrometer for prompt fission neutron spectrum measurements

This paper presents a digital neutron spectrometer based on simultaneous digitizing of the signals from the fission chamber and a scintillation detector. The scintillation detector is based on stilbene crystal. The intrinsic detection efficiency of the used stilbene crystal and the energy dependence of the light output for the recoil protons were measured. It is shown, that the method allowed us to achieve time resolution of 1.5 ns and an good n/γ separation down to neutron energies of 400 keV

Digital spectrometer for prompt fission neutron spectrum measurements

This paper presents a digital neutron spectrometer based on simultaneous digitizing of the signals from the fission chamber and a scintillation detector. The scintillation detector is based on stilbene crystal. The intrinsic detection efficiency of the used stilbene crystal and the energy dependence of the light output for the recoil protons were measured. It is shown, that the method allowed us to achieve time resolution of 1.5 ns and an good n/γ separation down to neutron energies of 400 keV

Legal regulation of the protection of the rights of abducted children (experience of France, Finland, and Sweden)

The purpose of the research is to analyze the law enforcement practice of protecting the rights of abducted children by the example of several European countries (France, Finland, and Sweden) and to highlight the main trends in the development of this practice at the international level. Research methods: analysis, synthesis, interpretation, structural-functional approach, classification, synthesis and interpretation of the information obtained. The main method of analysis of documents which are normative legal acts in the field of protection of the rights of abducted children at the level of international legislation, as well as the national legislation of France, Finland, and Sweden. The main result of the research was an analysis of the main national regulations on the protection of the rights of abducted children in France, Finland, and Sweden, as well as the activities of the central authority for international child abduction in these countries. In addition, the main trends in the development of law enforcement practice in the protection of the rights of abducted children at the international level were highlighted, using the examples of the countries studied. Scientific novelty of the research is substantiated by the determining role of The Hague Conventions of 1980 and 1996 as fundamental international documents in the field of international child abduction, the definition of the fundamental principle in the formation of national legislation and resolving the issues of protecting the rights of abducted children, and identifying the specifics of law enforcement practice in protecting the rights of abducted children in individual countries (France, Finland, and Sweden)

Magnetic Resonance Imaging of Changes in the Brain of Children Cured of Acute Lymphoblastic Leukemia

This study was aimed to systematize magnetic resonance imaging (MRI) presentation of toxic leukoencephalopathy, to find the correlation between method of central nervous system (CNS) leukemia prevention and changes on MRI, to find relationship between existence leukoencephalopathy on imaging and neurocognitive deficits in pediatric patients after anti-leukemic therapy. Brain MRI data of 48 children, who underwent a therapy course according to the ALL-MB intermediate risk protocol, was evaluated. In accordance with two arms of this protocol, they received either radiation therapy, or additional intrathecal administration of chemotherapeutic agents as a prevention of CNS leukemia. Also, neurocognitive tests were performed. According to the results of the performed investigation, 10 (50%) out of 20 children, who received cranial irradiation and 18 (66.6%) out of 27 patients, who received only intrathecal therapy demonstrated abnormal brain changes (leukoencephalopathy) according to MRI data. Leukoencephalopathy was mostly presented by diffuse zones and localized predominantly in the frontal and temporal lobes. There was no correlation between method of CNS prevention and the existence of leukoencephalopathy on MRI. The analysis of our data did not show significant differences in brain damage and severity of cognitive impairment depending on the type of prevention of CNS leukemia. Moreover, in this study no statistical correlation was found between leukoencephalopathy on MRI and neurocognitive impairment according to clinical tests data. Further long-term prospective studies and examinations should be performed to assess late neurotoxic effects