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Primary immunodeficiencies (PID) are genetically determined defects of the immune system. Despite significant advances in diagnosis and treatment of this group of disorders, personalized rehabilitation therapy aimed at improving the quality of a patient’s life (QOL) is not standardized. Our study of the rehabilitation effectiveness in a group of PID patients (n = 78; 59 boys and 19 girls), treated at the Russkoe Pole Rehabilitation Center, demonstrated significant improvement of the QOL in all aspects. The total QOL scale score increased from 66.13 to 74.89 points according to a child form and from 65.37 to 70.86 points according to a parent form. The greatest improvement in the QOL was achieved in children under 12 years of age, with an increase in the total scale score from 63.22 to 74.95 points (child form), and from 63.24 to 71.34 points (parent form). Therefore, personalized rehabilitation therapy can improve the QOL of patients with PID and can be applied in various rehabilitation centers. 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.

Annotation. The authors' study highlights the urgent problem of the development of toxic damage to the dentition under the influence of anticancer therapy in children and adolescents. The aim of the study was to study the violation of the homeostasis of the oral fluid and the severity of the carious process. Materials and methods. As part of a pilot study at the Russian Field Treatment and Rehabilitation Center, 63 children were studied the severity of mineral homeostasis disorders and damage to the dentoalveolar system. Results. In 67% of patients in the main group, there was an increased tendency to form carious cavities. 54.5% of them have complicated caries. One third of patients had more than five carious teeth. In the control group, carious lesions of the teeth in only three patients (20%), with the involvement of 1-3 teeth in the pathological process. Violation of enamel formation was diagnosed in 36% of children of the main group. There is a tendency for the accumulation of the studied microelements in the oral fluid, while in the blood serum there is a normal or insignificant decrease in their content. According to the literature, this may indicate destruction in the oral cavity. Deviations in the homeostasis of chemical elements in children who completed the treatment of malignant neoplasms and children in the control group were revealed. An increased content of osteotrophic microelements was found to correlate with the destructive processes of the teeth. Conclusion. It seems appropriate to study the severity of morphological changes in hard tissues of teeth and the electrolyte composition of mixed saliva in children and adolescents who have been cured of malignant neoplasms. The results of studies of gastric cancer, as an indicator of metabolic disorders, will make it possible not only to reveal the imbalance of macro- and microelements, but also to establish the effectiveness of adaptation mechanisms aimed at normalizing the elemental composition of the oral fluid.

Relevance.The relevance of the literature review presented by the authors is due to the diversity and complexity of the differential diagnosis of tumors of the orofacial zone in children and adolescents. Against the background of the absolute predominance of benign neoplasms, about 10-20% falls on the share of malignant neoplasms in this area. In this regard, polyclinic specialists often do not show sufficient oncological alertness, which leads to an unjustified lengthening of the diagnostic period and late diagnosis of malignant neoplasms.The purposeof the literature review is to discuss the results of studies on the epidemiological, clinical and therapeutic features of the tumor process in the orofacial zone in children and adolescents.Materials and methods.The searching of publications on the subject of the review were performed in the databases: https://www.ncbi.nlm.nih.gov/, https://elibrary.ru/cit_title_items.asp, https://www.researchgate.net/, https://elibrary.ru/. The authors describe the clinical manifestations of tumors depending on the location of the lesion and histological affiliation. The initial symptoms of both malignant and benign neoplasms are often nonspecific. Prevailing benign neoplasms can only be treated by surgery. Much less often in children and adolescents, malignant neoplasms are also found: squamous cell carcinoma of the oral cavity, Langerhans cell histiocytosis and others, which are treated in accordance with the principles of complex / combined anticancer therapy, including courses to minimize the amount of rehabilitation. Results.Timely diagnosis and prevention of the development of neoplasms in the orofacial area can reduce the severity of morphological and functional disorders in children and adolescents. Despite the use of effective methods of surgical or combination therapy, many need rehabilitation measures.Conclusions.The optimal position of a pediatrician, therapist, dentist, or surgeon at the stage of tumor diagnosis should be the implementation of oncological alertness, which implies an active approach without long-term "dynamic observation" of patients. Oncological alertness, especially among dentists, will improve the results of antitumor therapy in patients with Orofacial tumors.

The aim of this research was to analyze the effect of soil moisture regime on the intensity of 137Cs transfer into cereals using spring wheat as a model species. Accumulation of 137Cs in shoots of spring wheat grown on soil substrates differ by water and nutrients content was analyzed. The investigated water regimes were 40 % (insufficient moisture supply), 70 % (optimal moisture supply) and 85 % (excessive moisture supply) of full moisture capacity of the soils; substrates with optimal and low content of essential mineral nutrients were used in the experiment. The plants were grown in a chamber with controlled climate conditions. Increasing water content in the soil from 40 to 85 % of the total moisture capacity significantly reduces the 137Cs content in plants. The shape of the relationship between soil moisture and the soil-to-plant transfer of 137Cs depends on the agrochemical characteristics of the soil. The difference in the radioisotope accumulation in the shoots of wheat grown on extreme water regimes is about four times when substrate with optimum content of nutrients was used. The difference is less than two times when substrate with a low content of essential nutrients was used; moreover, there are no differences between the 70 and 85 % variants on a substrate with low content of nutrients. The revealed dependencies cannot be explained by the change in the share of bioavailable forms of 137Cs in the soil since it does not significantly change in soils with different moisture regimes.

The purpose of the work is to study the peculiarities of the elementary status of the children of Chelyabinsk region, living in the settlements similar in geochemical features, but various in the level of anthropogenic load and determine a major factor of mineral metabolism provision. The results of the study of hair trace elements content in 235 pupils of secondary school aged from 7 till 18 years are presented. All of them lived in the towns: Varna, Karabash and Tomino (Chelyabinsk region), located near the copper-porphyry ores, where commercial development of minerals was carrying out for a long time. The elementary status (40 chemical elements) was estimated by methods ICP-AS and ICP-MS in the Center of Biotic medicine (Moscow). Statistical processing of the results was carried using a program STATISTICA 8.0. It was established that irrespective of the place of residence for the examined children the deficiency of Se, Co, Cr was revealed, that was probably bounded to geochemical features of the territory of accommodation. The greatest impact on the elementary status was exerted by the level of anthropogenic loading. Children from Karabash had considerable excess of the recommended As and Pb values content in the hair; also they had the maximal, in comparison with other settlements, hair Cd, Be, Tl, Ba, Ag, Ni, Sn and Sb content. Children from this settlement had low Ca, Mg, P, B, Sr (the main osteotropic chemical elements) content in hair. Larger accumulation of Cu and Ni in hair of children from Karabash was connected with the ecological situation in the settlement and caused by emissions of Karabash copper-smelting plant.

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.

Characteristics of CD34+ cells mobilization were studied in children suffering with various diseases, like as their dependence on type and extent of inflammatory response. It was revealed that the children with signs of systemic inflammatory response syndrome exhibit a significant increase of absolute content and percentage of circulating CD34+ cells, whereas children with frequent infections, and with clinical signs of allergy during recurrence of their chronic allergic disease showed a significant decrease in CD34+ cell percentage. Meanwhile, children with allergoses, and those with common recurrent infectious states did not reveal any deviations in CD34+ cells from normal values.

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 neurochemical (neuropeptide) correction of disturbed automatic functions is a topical problem of modern neurophysiology and medicine. Most of the experimental studies on vegetative and motor reactions and the possibilities to compensate for their disturbance have been performed on partially immobilized animals [4, 5, 7, 8]. Only a few complex studies on vegetative and motor reactions under the conditions of free behavior have been conducted. We did not find publications on such studies on insectivores. Thyroliberin (thyrotropin-releasing hormone) is interesting because of its pronounced cardiovascular effect [1, 2]. This hormone attracts special attention as a specific regulator of respiration. Currently, thyroliberin is used in clinical practice to relieve hypoxia and normalize respiration and blood circulation in newborns [1, 2, 11‐13]. Therefore, we studied the role of thyroliberin in the regulation of conditioned motor and vegetative reactions in hedgehogs. The experiments were carried out on 13 hedgehogs Erinaceus europaeus. We used the model of eating behavior with simultaneous recording of the motor, respiratory, and cardiac components of higher nervous activity (HNA) in free-moving animals. An advantage of this method is that it allows the researcher to monitor the ratio between the three components of HNA at different stages of the formation of conditioned reflexes and to study the effects of neuropeptides on these components in animals belonging to lower stages of mammalian evolution. The electrocardiogram (ECG) was recorded with the use of silver cuff-shaped electrodes attached to three limbs (the two fore and the left hind). The respiratory component was recorded with the use of a nipple tube filled with graphite powder and set in a special cuff, which was attached to the hedgehog’s abdomen. To avoid noise when recording the vegetative activity in free-moving animals, we (together with V.P. Ivanov, an engineer at our laboratory) developed and built an operational amplifier with a high input resistance in the cardiograph. The noise caused by the effect of the supply-line frequency on the ECG record was eliminated with the use of special filters and shielded wires. Taking into account that hedgehogs would curl up into a ball, we used the following experimental procedure. Hedgehogs deprived of food (for one or two days) with the cuffs attached to them were taught to receive food reinforcement from the feeder (five to seven days). After this, conditioned reflexes were formed in the animals. A 50-dB, 200-Hz sound above the human hearing threshold served as a conditioned stimulus, and a 50-Hz sound, as a differentiation stimulus. Vegetative parameters were recorded on an N338-6P six-channel automatic recorder. In addition to positive conditioned responses, an internal inhibition of the extinction and differentiation types was formed in hedgehogs. We used an acute extinction, with an absence of motor responses to three consecutive nonreinforcements serving as a criterion for extinction. We measured the background heart rate (HR) and respiration rate (RR), their changes in response to the conditioned stimulus at different stages of the formation of the conditioned reflex, latent periods (LPs) of the motor and vegetative components, intersignal activity, and changes of these parameters after thyroliberin administration. The thyroliberin was prepared at the Department of the Chemistry of Natural Compounds, Faculty of Chemistry, St. Petersburg State University. The preparation was administered subcutaneously at a dose of 35‐40 μ g/kg 10 min before the experiment. We found that conditioned motor food-procuring responses were the first to be formed and reinforced: this took 60‐65 simultaneous applications of the conditioned and unconditioned stimuli. At the stage of reinforced conditioned responses, their latent period was relatively constant; its duration was 2.5 ∠ 0.53 s. Conditioned respiratory responses were formed later, after 75‐80 applications of the stimuli. At the initial stages, there were no constant patterns of conditioned respiratory responses. At the stage of reinforcement, the conditioned-response frequency tended to decrease. It took considerably more time for the cardiac component to PHYSIOLOGY