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Epidermolysis bullosa is a severe hereditary disease caused by mutations in genes encoding cutaneous basement membrane proteins. These mutations lead to dermal-epidermal junction failure and, as a result, to disturbances in the morphological integrity of the skin. Clinically, it manifests in the formation of blisters on the skin or mucosa that in some cases can turn into non-healing chronic wounds, which not only impairs patient's quality of life, but also is a live-threatening condition. Now, the main approaches in the treatment of epidermolysis bullosa are symptomatic therapy and palliative care, though they are little effective and are aimed at reducing the pain, but not to complete recovery. In light of this, the development of new treatment approaches aimed at correction of genetic defects is in progress. Various methods based on genetic engineering technologies, transplantation of autologous skin cells, progenitor skin cells, as well as hematopoietic and mesenchymal stem cells are studied. This review analyzes the pathogenetic methods developed for epidermolysis bullosa treatment based on the latest achievements of molecular genetics and cellular technologies, and discusses the prospects for the use of these technologies for the therapy of epidermolysis bullosa.

В монографии «Финансовая грамотность и финансовое просвещение населения: атлас российских практик» представлен спектр различных практик в области финансового просвещения граждан в регионах России. Путем анализа регионального опыта в области повышения финансовой грамотности были аккумулированы актуальные, представляющий высокий научно-прикладной интерес и значимость, практики, для дальнейшего осмысления и определения лучшего, эффективного опыта с целью его масштабирования и совершенствования. Материалы монографии представляют интерес для научных работников, государственных органов власти, преподавателей высших учебных заведений, аспирантов и студентов, изучающих тематику финансовой грамотности и финансового просвещения населения.

In this paper, we present our contribution to ABAW facial expression challenge. We report the proposed system and the official challenge results adhering to the challenge protocol. Using end-to-end deep learning and benefiting from transfer learning approaches, we reached a test set challenge performance measure of 42.10%., Results on test dataset and acknowledgements were added

Congenital hyperinsulinusm is rare disease characterized high secretion of insulin by pancreatic beta cells leading to the development of hypoglycemia. Persistent and transient forms of hyperinsulinism are distinguished. Transient hyperinsulinism are the most common cause of severe hypoglycemia in newborns. The etiology of this disease is not known. There are risk factors for the development of transient hyperinsulinism: asphyxia at birth, prematurity, maternal diabetes, low or large weight by gestation. Hypoglycemia with hyperinsulinism is severe. Therefore, early diagnosis and therapy especially during the neonatal period, are necessary.The article describes 3 clinical cases of transient hyperinsulinism in children with different gestational age and concomitant pathology. All children recevied insulinostatic therapy with diazoxide with a positive effect: euglycemia without glucose requirement . In all children, therapy was completed subsequently. At the time of publication of the article, the physical and psychomotor development of children is normal.

The article highlights a number of scientific and practical issues of methodology for assessing economic damage. Special attention is paid to the damage caused by environmental violations associated with the long-term accumulation of pollution in uncontrolled territories. A number of world and Russian examples of large-scale environmental violations that caused enormous economic damage are given. The necessity of deepening and correcting a number of points in the analysis of ecological-economic and ecological-legal problems of damage assessment is emphasized.

The article presents informative analysis of the place and role of population quality in the country’s socio-economic development. The study has two major objectives: to determine the dependence of quality characteristics of the population on purely economic factors, and assess the extent and nature of the impact of the population quality on economic development. We present an extended author’s description of the category “population quality” which includes the following aspects: economic activity, demographic processes, physical health, cultural potential, social health, educational potential, attitude to the environment. Ten statistical indicators out of 63 were selected and substantiated using correlation analysis within these aspects describing quality characteristics of the population. The information framework of the research includes data from the Federal State Statistics Service on 83 constituent entities of Russia for 2014. Based on selected indicators we conducted cluster analysis which helped classify the regions including and excluding economic factors. The obtained results of regions’ grouping by quality characteristics of their population identified the impact of the following two factors on homogeneity of regions in clusters: economic development and geographical position. We provide a substantial analysis of the groups of regions; demonstrate their distinctive features and their strengths and weaknesses. In order to analyze the influence of population quality on the economic development we carried out regression analysis. The economic development was characterized by GRP per capita. The population’s quality indicators serve as regressors. The significant factors are GRP per employee and the level of economic activity. The calculations confirmed the hypothesis about the weak influence of social indicators of population quality on the economic development. This result in no way diminishes the importance of improving population quality insignificant for the economy; it once again emphasizes that, in addition to economic, there are other goals of human development