Search

Basal cell nevus syndrome (BCNS, OMIM 109400) is a familial cancer syndrome characterized by the development of numerous basal cell cancers and various other developmental abnormalities, including epidermal cysts of the skin, calcified dural folds, keratocysts of the jaw, palmar and plantar pits, ovarian fibromas, medulloblastomas, lymphomesenteric cysts, and fetal rhabdomyomas. BCNS shows autosomal dominant inheritance and is caused by mutations in the patched 1 (PTCH1) gene and the suppressor of the fused homolog (SUFU) gene. In a few cases, variants of patched 2 (PTCH2) have been found in patients who met the criteria for BCNS. In an investigation of 11 Hungarian families who fulfilled the diagnostic criteria for BCNS, whole-exome sequencing (WES) and multiplex ligation-dependent probe amplification (MLPA) identified two novel pathogenic variants (c.2994C>A; p.Cys998Ter and c.814_818del; p.Asn272SerfsTer11), one recently identified variant (c.1737_1745del p.Val580_Val582del), and three recurrent disease-causing variants of the PTCH1 gene with a diagnosis rate of 63.6%. Disease-causing variants were not found for the SUFU and PTCH2 genes. These applied methods could not fully elucidate the genetic background of all the BCNS cases that we investigated. To uncover the missing heritability of BCNS, whole-genome sequencing or an epigenetic approach might be considered in the future.

Since COVID-19 was confirmed in Bangladesh in March 2020, the government have enacted stringent measures to prevent the spread of the coronavirus, which has had a significant impact on people’s lives. Food consumption habits of consumers have shifted as a result of declining grocery shopping frequency, negative income shock, and food prices shooting up. This paper aims to explore Bangladeshi consumers’ buying behaviour in association with the stress generated from a food supply shortage during the COVID-19 pandemic and the post-outbreak perception of the food industry, using a dataset with 540 online samples collected between July and August 2021. A two-stage cluster sampling method and self-administrated questionnaire techniques were adopted for collecting the data during the third wave of COVID-19. Using partial least squares path modelling (PLS-PM) and multivariate multiple ordered logit regression (MVORD) to reveal the pertinent structure between all the blocks, this study provides two key findings. First, a higher intensity of COVID-19 impact translates into higher food stress associated with income reduction and higher food prices. Second, food stress directly affects consumer buying and consumption behaviour. We strongly recommend connecting consumers with local producers and collective use of shared warehouses through institutions, policies, and reforms to prevent disruption in the food supply chain and to keep food prices stable. Additionally, food producers, distributors, stakeholders, and policy planners should strengthen the food supply chain to stabilize food security.

The close development of the economic relations between China and Central and Eastern Europe (CEE) since 2012 has triggered the European Union’s criticism of China’s foreign policy towards Eastern European countries. The European Union believes that China’s investment growth has led to a governance crisis in CEE countries. Based on the global governance indicators of the World Bank and the outward foreign direct investment (OFDI) data of the Ministry of Commerce of China, this paper conducts a test using the panel data model and the regression discontinuity method. An imbalanced panel dataset is adopted, covering 16 CEE countries from 2000 to 2018. The empirical research results indicate that, representing a small proportion of the investment inflows to CEE countries, China is not yet able to exert a domination effect on the region, and China’s economic power is far less than the European Union’s regulatory influence. Furthermore, China’s share of the OFDI in CEE has a U-shaped effect on the regulatory quality of host countries, and the two have a mutually causal relationship. Specifically, the impact on the host country’s regulatory quality is first manifested in the Shanghai effect, and when China’s share reaches a certain level, it is manifested in the California effect. The U-shaped effect is associated with the strategic factors of CEE countries and China’s positive contribution to good global governance.

The business environment has become complicated—full of risk and uncertainty over and above companies’ control— therefore companies must find mechanisms to enhance their performance in the light of this instability. The Iranian market is one of the best examples of unstable markets because of its political and economic circumstances, despite this, the pharmaceutical industry in Iran is considered one of the best industries, which is still working efficiently. The aim of the study is to investigate the impact of strategic flexibility on the performance of Iranian SME pharmaceutical companies, by considering the effect of environmental uncertainty as a moderator. The study is a cross-sectional one. Primary data was collected from 113 companies by using an adopted questionnaire. The questionnaires were forwarded to managers at these companies, a purposive (selective) sampling technique was used to collect the data, and the total number of responses that were valid for statistical analysis was 228. The response rate was 67.25%. The results showed that strategic flexibility positively affects companies’ performance. Supply and demand uncertainty moderate the relationship between strategic flexibility and companies’ performance.

The purpose of our study is to investigate the impact of women and independent directors on corporate social responsibility and financial performance. We use the fixed effect regression model as a baseline methodology. The data set includes information from 2010 to 2019 regarding Chinese non-financial companies, from which we use yearly information. The RSK rating is used for the assessment of corporate social responsibility reporting, ranging from 0 to 100, and other data are taken from the China stock market and accounting research (CSMAR) database. We use a two-stage least square (TSLS) regression model to control the possible problem of endogeneity. The empirical results show that gender diversity in boards significantly and positively affects CSR reporting. We do not find an effect due to non-executive directors on CSR reporting. The presence of non-executive directors on a board is mostly trivial in the case of China, as they do not have much influence with regard to decision making, especially related to CSR reporting. The control variables, such as board size, board member meeting frequency and leverage, are also found to have a significant effect on CSR reporting. Therefore, our results add a new aspect to the emerging literature on CSR reporting, especially in China. Furthermore, our results are robust with regard to the alternative variables under consideration. Our study has important implications. Our research enriches the existing literature on CSR and highlights the importance of female and independent directors having an impact on decisions related to the increased reporting of CSR activities. Our study contributes to the existing literature by presenting a pioneering investigation of the effect of female and independent directors on CSR reporting, as well as shedding light on the relationship in the context of an emerging economy.

Today's agricultural management decisions impact food security and sustainable ecosystems, even when operating with back-to-basic operations. In such endeavors, policymakers usually need a quantitative tool, such as trade-offs margins, to effectively adjust resource consumption or production. This paper applies the weighted slack-based measurement (SBM-DEA) program to 136 developing countries’ agricultural performance. First, it finds the current agricultural efficiency and then makes marginal trade-offs on desirable-output variables (such as crop yield and forest area) to see the effective changes in undesirable-output (such as methane and nitrous oxide emissions). The results show that choosing effective marginal trade-offs does not deteriorate the relative efficiency of the decision-making units (DMUs) below the efficient frontier line. Thus, such a method enables the decision-makers to determine the best marginal trade-off points to reach the optimal efficiencies and decide which output factor needs special brainstorming to design effective policy.

The increase in domestic transportation in developing countries may adversely affect the energy efficiency of road transportation due to effective productivity and carbon dioxide emissions (CO2). When evaluating quantitatively the countries on the efficiency frontier, poor efficiency can still be seen sometimes due to the slack available in undesirable output measures. This paper uses desirable and undesirable output variables, such as passenger-kilometers (PKM), tones-kilometers (TKM), and carbon dioxide (CO2), to compute the weakly efficient decision-making units (DMUs). The data envelopment analysis (DEA) technology is used to assess the efficiencies of the decision-making units (DMUs), which are countries in our case. Then, the trade-off method with efficient binding surfaces is used to attain the optimal efficiencies of the weakly efficient DMUs. The marginal rates aid this trade-off analysis. Resultantly, such marginal trade-offs do not deteriorate the efficiency of the DMUs below the frontier line. We calculate the maximum change (margin) in a specific variable amount when another variable’s amount is changed. Thus, such a computation gives us different margins, with which each output variable can be a traded off to bring a DMU further toward the closest optimal point possible. The marginal trade-off can help the managers and policymakers in effective decision-making, and it is further recommended to address efficiency damages (by the undesired outputs).

Since the introduction of the measure of economic policy uncertainty, businesses, policymakers, and academic scholars closely monitor its momentum due to expected economic implications. The US is the world&rsquo, s top-ranked equity market by size, and prior literature on policy uncertainty and stock prices for the US is conflicting. In this study, we reexamine the policy uncertainty and stock price nexus from the US perspective, using a novel dynamically simulated autoregressive distributed lag setting introduced in 2018, which appears superior to traditional models. The empirical findings document a negative response of stock prices to 10% positive/negative shock in policy uncertainty in the short-run, while in the long-run, an increase in policy uncertainty by 10% reduces the stock prices, which increases in response to a decrease with the same magnitude. Moreover, we empirically identified two significant thresholds: (1) policy score of 4.89 (original score 132.39), which negatively explain stock prices with high magnitude, and (2) policy score 4.48 (original score 87.98), which explains stock prices negatively with a relatively low magnitude, and interestingly, policy changes below the second threshold become irrelevant to explain stock prices in the United States. It is worth noting that all indices are not equally exposed to unfavorable policy changes. The overall findings are robust to the alternative measures of policy uncertainty and stock prices and offer useful policy input. The limitations of the study and future line of research are also highlighted. All in all, the policy uncertainty is an indicator that shall remain ever-important due to its nature and implication on the various sectors of the economy (the equity market in particular).

This study empirically examines the nexuses between the natural resource rent and financial development in the context of the emerging economy of Pakistan, between 1984 and 2018, by subsuming the important role of institutional quality in this context under symmetric, asymmetric, and threshold settings. The literature to date provides no evidence on the asymmetric relationship between natural resource rent and financial development, and the moderation role of institutional quality in this connection. We show that natural resource rent negatively influences financial development, whereas institutional quality boosts financial development and positively moderates the relationship in the context of Pakistan. Also, we find a single significant threshold value of 3.097 above which the relationship of resource rent-finance turns nonlinear&mdash, as up to this threshold the coefficient is 3.228, which declines slightly to 2.804 above the threshold level. This implies that regulators should maintain at least an institutional quality level of up to 3.097 to experience the most desired financial benefits of the natural resource rent in Pakistan. Moreover, the results corroborate the existence of asymmetries in the relationship between the natural resource rent and financial development. This empirical evidence provides fresh insight for stakeholders regarding ambiguous natural resource rents and financial sector development nexuses and recommends that planning organs in Pakistan and other countries in a similar development cadre should use institutional quality as a tool to avoid the resource curse and view natural resources as a blessing rather than a curse.

In modeling the impact of sovereign credit rating (CR) on financial markets, a considerable amount of the literature to date has been devoted to examining the short-term impact of CR on financial markets via an event-study methodology. The argument has been established that financial markets are sensitive to CR announcements, and market reactions to such announcements (both upgrading and degrading) are not the same. Using the framework of an autoregressive distributed lag setting, the present study attempted to empirically test the linear and non-linear impacts of CR on financial market development (FMD) in the European region. Nonlinear specification is capable to capture asymmetries (upgrades and downgrades) in the estimation process, which have not been considered to date in financial market literature. Overall findings identified long-term asymmetries, while there was little evidence supporting the existence of short-term asymmetries. Thus, the present study has extended the financial market literature on the subject of the asymmetrical impact of a sovereign CR on European FMD and provides useful input for policy formation taking into account these nonlinearities. Policies solely based upon linear models may be misleading and detrimental.

In the era of industrial digitalization, companies are increasingly investing in tools and solutions that allow their processes, machines, employees, and even the products themselves, to be integrated into a single integrated network for data collection, data analysis, the evaluation of company development, and performance improvement. To study the impact of Industry 4.0 on the company we used Porter’s (1985) value chain model, which is particularly useful when paying particular attention to corporate areas which have a primary role in customer value creation. Since the primary impact of Industry 4.0 is perceived in value-creating processes, and has so far had the greatest transformative effect in this area, the model can be considered to be appropriate. The objective of our research is to discover how companies operating in Hungary interpret the phenomenon of Industry 4.0, what Internet of Things (IoT) tools they use to support their processes, and what critical issues they face during adaptation. We applied a dual methodology in our investigation: We sent an online questionnaire to manufacturing and logistical service companies to investigate the IoT tools they use, and the problems they face, and received 43 answers we could evaluate. We also conducted four expert interviews with manufacturing firms to get deeper insights into the application, critical issues and development phases of IoT tools. During our research, we found that the spread of real-time data across companies—given the availability of appropriate analytical tools and methods—can have a significant impact on the entire company. In the case of CPS (Cyber Physical System), CPPS and Big Data Technologies, companies using them have been evaluated as having a higher level of logistic service, more efficient processes with their partners, improved cooperation between certain logistic functions, and higher market and financial performance and competitiveness. Applying more efficient production processes, and achieving better productivity and economies of scale, might also result in increased economic sustainability. Furthermore, we have found that companies have started on the path to digital evolution, and investments of this type have already begun.

No consensus has been reached on the problem of solving resource depletion. A recognition of the fact that resources are not endless and the Earth is a finite globe reinforces the idea that the vision of continuous economic growth is not sustainable over time. The aim of this paper is to examine the efficacy of real prices as an indicator of metals and oil in consideration of growth tendencies in the Consumer Price Indexes. In addition, enhancing the current literature on commodity price interrelationships, the main contribution of this study is the substitution of different proxies in order to justify the effect of scarcity and crude oil changes on the examined metal group prices. In order to demonstrate the usefulness of scarcity as an indicator of real price deviations, the study has been conducted involving various non-renewable metals, i.e., copper, molybdenum, zinc, gold and platinum group metals. The real price indices and metal prices of the US market are constructed between 1913 and 2015. Moreover, additional econometric analyses are also carried out to discover whether prices of various metals associate with oil prices and scarcity, as the proxy of reserves-to-production ratio. The linear regression results seem to suggest that the effects of the R/P ratios are negatively correlated with each of the examined precious (gold, PGMs), mass consumable (copper, zinc) and doping agent (molybdenum) metals from 1991 to 2015. An increase in oil-prices is positively associated with the price levels of each non-renewable resource in the short-run. The findings of multivariate co-integration and Granger causality tests also suggest that pairwise and direct relationships among these variables seem to arise in the long-run. These findings indicate essential questions that must be addressed by future generations in order to appropriately solve scarcity problems.

In our paper we examine the economics of a technological process which utilizes the separated biogas plant digestate as the primary material and, as auxiliary material, the waste heat produced by the cogeneration process, to produce a marketable pellet which can be used in two ways (to supply soil nutrients and heat energy). Using multivariate linear regression model we developed an equation for the biogas yield from the modelled production recipe and expected nutrient pellet prices, and sensitivity analysis were also performed for the substrate dry matter content. We found that pellets can be produced at a cost of 88–90 EUR/ton with a 6 to 10% dry matter substrate content and that, primarily, sales of pellets for heating justify pelleting; producer’s own use and use for nutrient purposes can only be justified in exceptional cases. In the case of dry solid content above 5%, the process does not require the total amount of waste heat; some of this can be used to cover other heat requirements.

The development of the tourism sector has been a question of strategic importance for Hungary, a small, open economy with limited natural resources. At the same time, these efforts often generate considerable environmental conflicts, decreasing the sustainability of the environment. To understand the potential methods of sustainable tourism development, and to develop the optimal policy, it is essential to clarify the actors, their systems of interest and the potential ways of forging coalitions between them. The article presents an analysis of two case studies of rural tourism development: the “softening” of tourism at the most important touristic attraction in Hungary, Lake Balaton; and the conflicts arising from wine tourism development. Based on institutional economics, principle–agent theory and strategic management, and applying the MACTOR method, the authors identify the key actors, present the network of their mutual influences and goals, determine the most important conflicts and highlight the potential coalitions between them from the point of view of sustainable rural tourism development, as well as ways to further develop the regulatory environment. Based on this analysis, the article proves: (1) the importance of the modernization and re-organization of the public administration structure, focusing on optimal utilization of resources, as opposed to attaching to traditions; (2) the importance of forming clusters of different partners; (3) the strengthening of the knowledge base of decisions concerning sustainable tourism management; and (4) increasing conscious planning, based on the inclusion of different interest groups and long-term prognoses in local decision making, minimises the environmental burden of tourism.

The article analyses co-authorship and co-citation networks in Food Policy, which is the most important agricultural policy journal in the field of agricultural economics. The paper highlights the principal researchers in this field together with their authorship and citation networks on the basis of 714 articles written between 2006 and 2015. Results suggest that the majority of the articles were written by a small number of researchers, indicating that groups and central authors play an important role in scientific advances. It also turns out that the number of articles and the central role played in the network are not related, contrary to expectations. Results also suggest that groups cite themselves more often than average, thereby boosting the scientific advancement of their own members.

This article examines how worldwide governance, global competiveness, and other institutional determinants have influenced the number of accounting fraud cases in several countries. The researchers have focused more closely on the importance of ‘good governance’ as one of the indicators of development objectives in itself. The institutional perspective is employed to explain the complexity of frauds in different societies which can be compatible for the purposes of international judgments in order to increase the effectiveness of previous forensic accounting theories. In this paper, a linear regression model is tested where governance, competitiveness, and other institutional variables are associated with a measure of accounting fraud cases. From our results, we can merely claim that an increased level of controlled corruption and political stability might reduce the number of fraud cases in various countries, while more effective and independent governance services with a higher freedom of expression seemed to increase them. The existence of accounting crimes also appeared to be a suitable proxy of better competitiveness. Anglo-Saxon countries have more stated fraud cases than other countries, attributed, perhaps, to the finest commercial courts with the most professional and least corrupt judges in the world, with centuries of precedent cases and experience in dealing with fraud. Moreover, we believe that a better understanding of fraud detection is a potentially important element in forensic accounting analytics in the success of governance policies to enhance development and reduce the risk of bankruptcies related to the reported fraud cases of enterprises.

This article presents the history of integrated farming in aquaculture through a Hungarian case study. The development of Hungarian integrated aquaculture is aligned with global trends. In the previous millennium, the utilization of the nutrients introduced into the system was the main aspect of the integration. In Hungary, technologies that integrated fish production with growing crops and animal husbandry appeared, including for example: large-scale fish-cum-rice production; fish-cum-duck production; and integrated pig-fish farming which were introduced in the second half of the 20th century. Today, the emphasis is on integrating the use of the kind of feed where the main goal is to minimize nutrient loads in the surrounding natural ecosystems and to maximize the utilization of the unit’s water resources. The various modern integrated freshwater aquaculture systems, such as intensive fish production combined with wetland, recirculation aquaculture system and multi-functional aquaculture, have proved their viability. However, the future opportunities for these systems have not always been properly recognized and acknowledged when the future of European aquaculture is discussed.

This research focuses on the role of trust and the impact of its level on the revenue, earnings before tax and the degree of flexibility of logistics service providers (LSPs). More specifically, the role of the executive manager is examined in relation to the impact of business relationships (trust levels) within and between organizations. In addition, the analysis covers the development of revenue, earnings before tax and degree of flexibility of logistics service providers in the context of the role of the head manager. The data were collected from 51 logistics service providers in Hungary. The results show that the level of trust established in the organization (with the employees, co-workers etc.) has a positive impact on the earnings before tax. Furthermore, this paper confirms that the trust executive managers establish around them is an important performance factor which even consumers perceive and that it has major significance in terms of degree of flexibility. This research further increases our understanding of the role and importance of trust as a strategic success factor for LSPs.