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Introduction: Interferon I (IFN I) signaling hyperactivation is considered one of the most important pathogenetic mechanisms in systemic lupus erythematosus (SLE). Early manifestation and more severe SLE courses in children suggest a stronger genetic influence in childhood-onset SLE (cSLE). Aim: To evaluate IFN-I score and SLE-associated genetic variants in cSLE. Material and Methods: 80 patients with cSLE were included in the study. IFN I-score was assessed by real-time PCR quantitation of 5 IFN I-regulated transcripts (IFI44L, IFI44, IFIT3, LY6E, MXA1) in 60 patients. Clinical exome sequencing (CES) was performed in 51 patients. Whole-exome sequencing was performed in 32 patients with negative results of CES. Results: 46/60 patients (77%) had elevated IFN-I scores. Leucopenia and skin involvement were associated with over-expression of IFI44 and IFI44L, while hypocomplementemia—with hyperactivation of IFIT3, LY6E, and MX1. No correlation of IFN-I score with disease activity was found. At least one rare genetic variant, potentially associated with SLE, was found in 29 (56.9%) patients. The frequency of any SLE-genetic variants in patients with increased IFN scores was 84%, in patients with normal IFN scores—33%, and in the group whose IFN score was not assessed was 65% (p = 0.040). The majority of genetic variants (74%) are functionally related to nucleic acid sensing and IFN-signaling. The highest frequency of genetic variants was observed in Sakha patients (9/14; 64.3%); three and two unrelated patients had identical variants in PTPN22 and TREX1 genes, respectively. Conclusions: More than half of patients with childhood-onset SLE have rare variants in SLE-associated genes. The IFN-I score could be considered a tool for the selection of patients for further genetic assessment in whom monogenic lupus is suspected. [ABSTRACT FROM AUTHOR]

This article the article is concerned with a substantiation of procedures ensuring the implementation of statistical research and monitoring of investment development of the Russian regions, which would be pertinent for modern development of the state statistics. The aim of the study is to develop the methodological framework in order to estimate the investment development of regions of Russia, providing an increase in the usage of own resources and an achievement of the objectives of economic development. Basic principles of neo-Keynesian economic theory and methods of statistical analysis (index method, clustering method, taxonomic method) are the methodological basis of the research. Information basis includes analytical materials, expert opinions and statistical data of Federal State Statistics Service on the development of Russian regions. Results of the study: the structure of the investment development of a region as an object of statistical research and the system of statistical indicators characterizing its elements were elucidated; the algorithm of statistical research and monitoring system of investment development of regions of Russia were tested. Conclusions: proposed procedures allowed implementing a monitoring of investment development in Russian regions in 2005-2013. On its basis, Russian regions, which are "points of investment growth", and regions, which use investments for achieving development goals ineffectively were identified. This made it possible to develop and justify practical recommendations for optimizing the allocation of budget investments during the crisis in the Russian economy.

This is to study the methodological foundations of perceptual mapping in printing industry enterprises. This research has a practice focus which affects the choice of its methodological framework. The authors use such scientific research as analysis of cause-effect relationships, synthesis, problem analysis, expert evaluation and image visualization methods. In this paper, the authors present their assessment of the competitive environment of major printing industry companies in Kirov oblast; their assessment employs perceptual mapping enables by Minitab 14. This technique can be used by experts in the field of marketing and branding to assess the competitive environment in any market. The object of research is printing industry in Kirov oblast. The most important conclusion of this study is that in perceptual mapping, all the parameters are integrated in a single system and provide a more objective view of the company’s market situation.

In spite of many theoretical and methodological approaches and methods set forth by both domestic and Western scientists, issues of formation of an effective team are still quite challenging in terms of organizational efficiency improvement. The paper critically examines existing concepts and terms of formation of a new structure of management of an enterprise (organization), based on a hierarchical team approach. The main methods of the study of this problem are the abstract and logic, dialectic, intuitive and prophetic, deductive, based on religious and philosophical, and socio-economic, system-situational approaches, making it possible to holistically consider the processes of formation and establishment of the system of one-level team management, based on a team culture of thinking. The authors made a conclusion about the narrowness of all works on the team culture of organization management in the tideway of the Western liberal market economy, and their low efficiency in the context of Russian cultural identity and self-determination. The paper reveals the reasons of insufficient efficiency of existing hierarchical team system. Article information may be of practical value to a new generation of managers who will build a new socio-economic culture of thinking, based on a new type of economy: person-oriented one. [ABSTRACT FROM AUTHOR]