Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Three major objectives were proposed for the future development of CPGs: MCTD diagnosis (diagnostic criteria), MCTD initial and follow-up evaluations, MCTD treatment. Early diagnosis, epidemiological data, assessment of burden of disease and QOL aspects are among the unmet needs identified by patients.

The aim of the research is to address the challenges that may be a constraint on economic growth and development. In the research, we used the relevant literature and acts that were in front of the delegates at the National Assembly of Republic of Srpska. The research question is: "What are the main characteristics of the Republic of Srpska's debt?" In line with this, the methods employed in this paper are the analysis and synthesis of previous researchs, theoretical findings and publicly accessible documents pertaining to the debt of Republic of Srpska, and an overview of the case studies.  The contribution of the paper is reflected in the explained wideness and the possibilities of using different sources of money, and the limits on which the debts can be used in order to achieve sustainability tasks. We have concluded that this year, the Republic of Srpska bill has reached the largest amount of debt – KM 858 million (755 million principal and 103 million interest). In line with the presented evidence, priority should be given to projects that would be financed by advantageous credit arrangements and to rationalize public spending. Despite the usual opinion, we have proven that the debt of the Republic of Srpska is sustainable - the average interest rate is lower than the GDP growth rate and that, although unpopular, the currency board system corresponds to the position of Republic of Srpska. However, we have pointed out the danger of stereotype that the public debt of less than 60% necessarily indicates that the country is in a good position.

The aim of this paper is the analysis of the importance of the beverages sector and concrete trade facilitation measures for CEFTA 2006 economies, in general and for their intra-regional trade, in particular. The paper analyses both trade in beverages of CEFTA 2006 economies and heterogeneous group of barriers that exporters and importers are faced with at the CEFTA 2006 beverages market. Additionally, the paper points to possible solutions for elimination of those barriers through the implementation of selected trade facilitation measures. As the most of problems are related to the trade facilitation and marked as problems that could be easily removed, the most recommendations are based on the WTO Trade Facilitation Agreement measures.

Background To date there is scarce published evidence reporting the dual blood supply reaching anterior papillary muscle (APM), which descends from both major coronary arteries. Such a vascular configuration can prevent the dysfunction of right ventricular entire valvular system in case of the occlusion of proximal part of either right coronary artery (RCA) or left coronary artery (LCA). The aim of our study was to determine the vascular pattern of APM blood supply which originates from two main coronary arteries, in the context of the APM and septomarginal trabecula (SMT) topography. Methods The study was carried out using tissue obtained from 36 human hearts. The material was divided into four morphological types of SMT/APM arrangement. Vascularization and blood supply pattern of papillary muscle was investigated following the analysis of multiple tissue cross sections. The origin of APM arterial supply was traced back to both main coronary arteries. Cross-sectional area of the arteries was estimated at the base of APM and compared within mixed male-female population, aged 18–76. Results We noted that as much as 78% of entire APM material had a blood supply vasculature originating from both LCA and RCA branches. In contrast, 22% of cases APM was supplied by a single coronary artery, while in each case it proved to be LCA. We have never found APM arterial supply provided exclusively by RCA. In case of double AMP blood supply an average of total cross-section area of the arteries branching from LCA, was noted to be in excess of two and a half times bigger in type III and more than two times bigger in type IV, as compared with the arteries originating from RCA. Conclusions Our research confirm the possibility of double blood supply which vascularizes APM, but the finding does not necessarily apply in all cases. However, APM seems to be predominantly vascularized by arteries deriving from LCA, regardless of their morphological type.

The functioning of each traffic system depends to a great extent on the way the rail transport system operates. Taking into account the aspect of market turbulence and the dependence on adequate delivery when it comes to freight transport and traffic in accordance with a yearly Timetable in passenger traffic, transport policies are changing with time. Therefore, this document is considering the railway management models on the territory of Bosnia and Herzegovina. For the purpose of evaluating these models, a new hybrid model has been applied, i.e. the model which includes a combination of the Delphi, SWARA (Step-Wise Weight Assessment Ratio Analysis) and MABAC (Multi-Attributive Border Approximation Area Comparison) methods. In the first phase of the study, the criteria ranking was determined based on 16 expert grades used in the Delphi Method. After that, a total of 14 decision-makers determined the mutual criteria impact, which is a prerequisite for the application of the SWARA Method used to determine the relative weight values of the criteria. The third phase involves the application of the MABAC Method for evaluating and determining the most suitable variant. In addition, a sensitivity analysis involving the application of the ARAS, WASPAS, SAW and EDAS methods has been performed, thus verifying the previously obtained variant ranking.

High availability of automation systems is one of the main goals for the companies from all industrial branches. To achieve and maintain this high availability, the condition monitoring of the automation systems is an essential building block. However, as automation systems become increasingly equipped with numerous mechanical, electrical, and software components, creating a condition monitoring solution is becoming more and more challenging and requires knowledge from multiple engineering disciplines. Today, creating a condition monitoring solution is mostly based on the experience and preferences of the developers without a systematic and interdisciplinary method. Today, methods and tools supporting an interdisciplinary development exist. However, they do not fully consider condition monitoring relevant information. In addition, tools that increase software productivity and ease the adjustment of condition monitoring software are lacking. The main goal of this paper is to narrow the condition monitoring expertise gap by proposing convenient, systematic, and automated techniques to support the development of condition monitoring solutions from their design to their implementation. To achieve this goal, we propose an extension of the CONSENS systems engineering method to face issues caused in the design phase. By adopting a Model-Driven Development (MDD) approach, we propose a Domain-Specific Language (DSL) for condition monitoring that promotes increased understandability, and automation during the software implementation phase.

The fourth industrial revolution (Industry 4.0) strives for an optimized and flexible production process to reduce configuration costs. To achieve such a production process, first architecture-level decisions (i.e. deployment configuration, scheduling, etc.) are optimized. Following, these optimized architectural configurations are applied to dynamically reconfigure industrial control applications. To calculate software configurations, current research applies Design Space Exploration (DSE) techniques embedded into the IEC 61499 model-based approach. However, this research either applies simple and non-applicable constraints and objectives for real-life problems or considers architectural optimization solely at the design phase. Thus, reconfiguring industrial control applications is still an exhausting and manual task that requires production process' downtime. In this Ph.D. thesis, I handle the automatically optimized reconfiguration of the industrial automation systems. In particular, I propose applying DSE to calculate architectural configurations of IEC 61499-based control applications. To define different configuration problems from real-world situations, I identify domain-specific constraints and objectives. Furthermore, I will tackle the problem of runtime reconfiguration by applying several optimization strategies in various context scenarios and investigating an incremental search for new optimal configurations.