Search results for: rare disease policy

Authors: A. M. Bumbea, A. Musetescu, P. Ciurea, A. Bighea

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Objectives: The authors present a Parsonage Turner syndrome, a rare disease characterized by onset in apparently healthy person with shoulder and/or arm pain, sensory deficit, motor deficit. The causes are not established, could be determinate by vaccination, postoperative, immunologic disease, post traumatic etc. Methods: The authors present a woman case, 32 years old, (in 2006), no medical history, with arm pain and no other symptom. The onset was sudden with pain at very high level quantified as 10 to a 0 to 10 scale, with no response to classical analgesic and corticoids. The only drugs which can reduce the intensity of pain were oxycodone hydrochloride, 60 mg daily and pregabalinum150 mg daily. After two weeks the intensity of pain was reduced to 5. The patient started a rehabilitation program. After 6 weeks the patient associated sensory and motor deficit. We performed electromyography for upper limb that showed incomplete denervation with reduced neural transmission speed. The patient receives neurotrophic drugs and painkillers for a long period and physical and kinetic therapy. After 6 months the pain was reduced to level 2 and the patient maintained only 150 mg pregabalinum for another 6 months. Then, the evaluation showed no pain but general amiotrophy in upper limb. Results: At the evaluation in 2009, the patient developed a rheumatoid syndrome with tender and swelling joints, but no positive inflammation test, no antibodies or rheumatoid factor. After two years, in 2011 the patient develops an increase of antinuclear antibodies. This context certifies the diagnosis of lupus and the patient receives the specific therapy. Conclusions: This case is not a typical case of onset of lupus with PTS, but the onset of PTS could include the onset of an immune disease.

Keywords: lupus, arm pain, patient, swelling

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Authors: Desiree Vandenberghe, Johan Albrecht

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Non-communicable diseases (NCDs) are responsible for the vast majority of deaths in the European Union (EU) and represent a large share of total health care spending. A future increase in this health and financial burden is likely to be driven by population ageing, lifestyle changes and technological advances in medicine. Without adequate prevention measures, this burden can severely threaten population health and economic development. To tackle this challenge, a correct assessment of the current burden of NCDs is required, as well as a projection of potential increases of this burden. The contribution of this paper is to offer perspective on the evolution of the NCD burden towards the future and to give an indication of the potential of prevention policy. A Non-Homogenous, Semi-Markov model for the EU was constructed, which allowed for a projection of the cost burden for the four main NCDs (cancer, cardiovascular disease, chronic respiratory disease and diabetes mellitus) towards 2030 and 2050. This simulation is done based on multiple baseline scenarios that vary in demand and supply factors such as health status, population structure, and technological advances. Finally, in order to assess the potential of preventive measures to curb the cost explosion of NCDs, a simulation is executed which includes increased efforts for preventive health care measures. According to the Markov model, by 2030 and 2050, total costs (direct and indirect costs) in the EU could increase by 30.1% and 44.1% respectively, compared to 2015 levels. An ambitious prevention policy framework for NCDs will be required if the EU wants to meet this challenge of rising costs. To conclude, significant cost increases due to Non-Communicable Diseases are likely to occur due to demographic and lifestyle changes. Nevertheless, an ambitious prevention program throughout the EU can aid in making this cost burden manageable for future generations.

Keywords: non-communicable diseases, preventive health care, health policy, Markov model, scenario analysis

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Authors: Purabi Saikia

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The present study was carried out across 84 vegetated grids (>10% vegetation cover) along an urbanization gradient, ranging from the urban core to peri-urban and natural vegetation in and around Ranchi, Eastern India, aiming to examine the phytosociological attributes by belt transect (167 transects each of 0.5 ha) method. Overall, plant species richness was highest in natural vegetation (242 spp.), followed by peri-urban (198 spp.) and urban (182 spp.). Similarly, H’, CD, E, Dmg, Dmn, and ENS showed significant differences in the tree layer (H’: 0.45-3.36; CD: 0.04-1.00; E: 0.25-0.96; Dmg: 0.18-7.15; Dmn: 0.03-4.24, and ENS: 1-29) in the entire urbanization gradient. Various α-diversity indices of the adult trees (H’: 3.98, Dmg: 14.32, Dmn: 2.38, ENS: 54) were comparatively better in urban vegetation compared to peri-urban (H’: 2.49, Dmg: 10.37, Dmn: 0.81, ENS: 12) and natural vegetation (H’: 2.89, Dmg: 13.46, Dmn: 0.85, ENS: 18). Tree communities have shown better response and adaptability in urban vegetation than shrubs and herbs. The prevalence of rare (41%), very rare (29%), and exotic species (39%) in urban vegetation may be due to the intentional introduction of a number of fast-growing exotic tree species in different social forestry plantations that have created a diverse and heterogeneous habitat. Despite contagious distribution, the majority of trees (36.14%) have shown no regeneration in the entire urbanization gradient. Additionally, a quite high percentage of IUCN red-listed plant species (51% and 178 spp.), including endangered (01 sp.), vulnerable (03 spp.), near threatened (04 spp.), least concern (163 spp.), and data deficient (07 spp.), warrant immediate policy interventions. Overall, the study witnessed subsequent transformations in floristic composition and structure from urban to natural vegetation in Eastern India. The outcomes are crucial for fostering resilient ecosystems, biodiversity conservation, and sustainable development in the region that supports diverse plant communities.

Keywords: floristic communities, urbanization gradients, exotic species, regeneration

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Authors: Blen Telayneh Melese

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Rural development, the unending quest of a developing country, cannot be succeeded in deprived of human capital development. Human capital, the economic pillars of a country's development, appeals a policy-based supports while fulfilling what is expected. Ethiopia, one of the rural countries with untouched and forgotten land and human force, owes historical experiences of educational policy intending for mobilization of its citizen for the advancement of the overall economy. Rural Ethiopia as well has been the focus of those educational policies, considering the economic resources entrenched with in. In this literature review paper, Ethiopian educational policy and its contribution to human capital development, as well as its role in generating quality human labor force, is assessed concisely. The author argues that the foundation of rural development such as technology, knowledge, infrastructure, market chain, communication and etc., can only be achieved through enhanced education policy that conciliates the existing reality of rural communities. Ethiopia still needs an education policy that enables it to generate a human capital that is oriented with the rural areas economic opportunities and challenges.

Keywords: Ethiopia, rural development, human capital development, education policy

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Authors: Umang Prajapati

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Elected prime minister of India Narendra Modi has very largely focused on improving ties with the neighbors since day one of his regime. This was the most significant initiative to focus on major Asian powers also emphasizing on the two decades old look east policy. The “neighborhood first policy” as termed by the media has been a corner stone in improving ties with the immediate neighbors of the country through several bilateral talks with the nations individually. However, the announcement of demonetisation policy in India, ceasing the usage of 500 and 1000 rupee notes has rattled countries like Nepal, Bangladesh, Bhutan, Sri Lanka and Myanmar who encourage Indian currency parallel to theirs. According to the ministry of commerce and industry (MCI), India’s total trade with neighboring countries stood at US$21.6 in the fiscal year 2015-16, India has good surplus trade surplus with its neighbors and has a strong interest in ensuring smooth trade flows. India might have this benefit, but yet this policy can create issues between India and neighboring countries. The demonetisation policy might benefit in the long run, but in the short run, this might create border issues. While there would be more countries affected by this policy, this paper will emphasize on the problems faced by the countries and the impact of demonetisation on all other neighboring countries.

Keywords: bilateral trades, demonetization, neighborhood policy, value of rupee

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Authors: Sunita Patel

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Cardiovascular diseases are the leading cause of morbidity as well as mortality in India. Objective of this study is to examine CVDs prevalence and identify their behavioral and cultural risk factors with the help of SAGE-2007 data conducted on 6th states in India. Findings reveal that 18.3% of people diagnosed with CVDs in India. Higher disease occurs in an increasing rate between ages of 30-39 having OR 2.45 (CI: 1.66-3.63) and 70+ age OR 7.45 (CI: 4.82-11.49) times higher compare to 18-29 age group respectively. Wealth quintile higher CVD occurs as 3rd in 60% (CI: 1.16-2.21) and in richest 5th quintile 58% (CI: 1.13-2.21) contrast to lowest quintile. Relative risk depicted that 22.4% in moderate and 44% in vigorous activity have less chance of diseases compare to who performed no work and those who consumed alcohol. Results reveal that policy prospect should be recommended and that it would be beneficial for awareness of people and their future.

Keywords: behavioral risk, cultural risk, cardio-vascular diseases, wealth quintile

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Authors: Jeonghwan Jeon

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As the science and technology (S&T) has been rapidly advanced, the national government attempts to reflect changes in the S&T for promoting public R&D activities and economic development. Amongst others, due to the rapid advances and changes of S&T, it becomes important to analyze the trends of S&T policies for formulating the new policy and investigating promising S&T fields. Thus, this paper aims to trace the national S&T policies during this decade for analyzing the change of major S&T fields in the case of South Korea. As one of the organization for S&T policy in South Korea, the National Science and Technology Council (NSTC) has been established to coordinate inter-ministerial policies and programs and to determine all of the national and public S&T policy of South Korea. In this regard, the items on national S&T policy determined by the NSTC are useful for understanding the needs for major S&T fields and adapting to the rapid change of S&T. To this end, we first gathered the data on 512 items on the S&T agenda from 1999 to 2013. Based on these items, the trend of S&T policies is monitored and the major S&T fields are derived. Differences of policy purposes between S&T fields are identified to provide guideline for policy making such as budget allocation or investment promotion as well.

Keywords: national science and technology, policy, trends, S&T field

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Authors: Nurudeen Oluwasola Lasisi, Abdulkareem Afolabi Ibrahim

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Newcastle disease is an infection of domestic poultry and other bird species with virulent Newcastle disease virus (NDV). In this paper, we study the dynamics of modeling the Newcastle disease virus (NDV) using a novel quarantine-adjusted incidence. We do a comparison of Vaccination, linear incident rate, and novel quarantine adjusted incident rate in the models. The dynamics of the models yield disease free and endemic equilibrium states. The effective reproduction numbers of the models are computed in order to measure the relative impact for the individual bird or combined intervention for effective disease control. We showed the local and global stability of endemic equilibrium states of the models, and we found that stability of endemic equilibrium states of models are globally asymptotically stable if the effective reproduction numbers of the models equations are greater than a unit.

Keywords: effective reproduction number, endemic state, mathematical model, Newcastle disease virus, novel quarantine-adjusted incidence, stability analysis

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Authors: Jeonghwan Jeon

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As the science and technology(S&T) has been rapidly advanced, the national government attempts to reflect changes in the S&T for promoting public R&D activities and economic development. Amongst others, due to the rapid advances and changes of S&T, it becomes important to monitor the trends of S&T policies for formulating the new policy and investigating promising S&T fields. Thus, this paper aims to trace the national S&T policies during this decade for monitoring the change of major S&T fields in the case of South Korea. As one of the organization for S&T policy in South Korea, the National Science and Technology Council (NSTC) has been established to coordinate inter-ministerial policies and programs and to determine all of the national and public S&T policy of South Korea. In this regard, the items on national S&T policy determined by the NSTC are useful for understanding the needs for major S&T fields and adapting to the rapid change of S&T. To this end, we first gathered the data on 512 items on the S&T agenda from 1999 to 2013. Based on these items, the trend of S&T policies is monitored and the major S&T fields are derived. Differences of policy purposes between S&T fields are identified to provide guideline for policy making such as budget allocation or investment promotion as well.

Keywords: science and technology policy, trends, S&T field, monitoring

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Authors: Robert Gilliland

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In any organization, a potential issue can arise and become a problem when management deviates from the standard norms set in the system development process of an IT system and the policies that pertain to it. In these instances, cybersecurity is a big challenge that organizations have to face in safeguarding the data that they generate and use. When a new idea, task, or process begins, specific standards must be followed, along with the policies and procedures that ensure the safeguard of data in the information system within the company. A good IT Strategy and Policy should have individuals who are in charge of overseeing the design, development, implementation, and auditing of these policies. Auditors are people who check to make sure that the issue conforms with the plan that is in place. Management has the ability through the role of the manager to potentially abuse power is given and to direct specific ideas, events, projects, and outcomes that are contrary to the vision or goals of the company.

Keywords: strategic policy, policy management, new policy, strategic planning

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Authors: Vera Varazashvili, Murman Tsarakhov, Tamar Mirianashvili, Teimuraz Pavlenishvili, Tengiz Machaladze, Mzia Khundadze

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Standard Gibbs energy of formation ΔGfor(298.15) of lanthanide-iron double oxides of garnet-type crystal structure R3Fe5O12 - RIG (R – are rare earth ions) from initial oxides are evaluated. The calculation is based on the data of standard entropies S298.15 and standard enthalpies ΔH298.15 of formation of compounds which are involved in the process of garnets synthesis. Gibbs energy of formation is presented as temperature function ΔGfor(T) for the range 300-1600K. The necessary starting thermodynamic data were obtained from calorimetric study of heat capacity – temperature functions and by using the semi-empirical method for calculation of ΔH298.15 of formation. Thermodynamic functions for standard temperature – enthalpy, entropy and Gibbs energy - are recommended as reference data for technological evaluations. Through the isostructural series of rare earth-iron garnets the correlation between thermodynamic properties and characteristics of lanthanide ions are elucidated.

Keywords: calorimetry, entropy, enthalpy, heat capacity, gibbs energy of formation, rare earth iron garnets

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Authors: Muhandiram Rallage Ruvini Nisansala Yatigammana, Anuruddhika Kumudu Kumari Rajakaruna Jayathilaka

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Sports injuries are common among teenagers and children engaged in organized sports. While most sports injuries are typical, some rare occurrences involve conditions such as eye, dental, cervical, and rare internal organ injuries, such as pancreatic injuries. These injuries, especially traumatic pancreatitis, require prompt attention due to their potential for severe and sometimes fatal complications. This case revolves around a real accident involving a 12-year-old girl, Piyumi, who suffered a face-to-face collision during netball practice, resulting in severe abdominal pain. After a medical examination, she was diagnosed with a rare pancreatic injury, uncommon in children compared to adults. In Piyumi’s case, she had a grade 3 pancreatic injury and underwent non-surgical management, successfully healing her wound without surgery. The study attempts to fill empirical and population gaps, addressing a rarely discussed injury experienced by a 12-year-old female netball player. The paper will also provide an in-depth understanding of pancreatic injury, which is a rare sports injury. The study’s main objective was to investigate the incidence and characteristics of pancreatic injury, particularly focusing on pancreatic trauma, among children and adolescents engaged in high-impact sports, such as netball. This research adopted a case study strategy, employing interviews as the primary data collection method. Interviews were conducted with Piyumi, her parents, and the two specialist doctors directly involved in her treatment, providing firsthand accounts and insights. By examining the case, the paper arrives at three main conclusions. Firstly, pancreatic damage is uncommon, especially in the sports world, and proper diagnosis is essential to avoiding health concerns, particularly for minors. Secondly, CT (Computed Tomography) was useful in locating the injury, as injuries can be diagnosed very well with Computed Tomography (CT) images. Finally, and most importantly, pancreatic injuries are infrequent, but trauma can still occur, particularly in high-impact sports or accidents involving extreme force or falls. These injuries should be accurately diagnosed and treated promptly.

Keywords: child athlete, pancreatic injury, rare sports injuries, sportswoman

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Authors: Vera Varazashvili, Murman Tsarakhov, Tamar Mirianashvili, Teimuraz Pavlenishvili, Tengiz Machaladze, Mzia Khundadze

Abstract:

Standard Gibbs energy of formation ΔGfor(298.15) of lanthanide-iron double oxides of garnet-type crystal structure R3Fe5O12 - RIG (R – are rare earth ions) from initial oxides are evaluated. The calculation is based on the data of standard entropies S298.15 and standard enthalpies ΔH298.15 of formation of compounds which are involved in the process of garnets synthesis. Gibbs energy of formation is presented as temperature function ΔGfor(T) for the range 300-1600K. The necessary starting thermodynamic data were obtained from calorimetric study of heat capacity and by using the semi-empirical method for calculation of ΔH298.15 (formation). Thermodynamic functions for standard temperature – enthalpy, entropy and Gibbs energy - are recommended as reference data for technological evaluations. Through the isostructural series of rare earth-iron garnets the correlation between thermodynamic properties and characteristics of lanthanide ions are elucidated.

Keywords: calorimetry, entropy, heat capacity, Gibbs energy of formation, rare earth iron garnets

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Authors: Mahmoud Hassan

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Understanding the impact of market- and non-market-based environmental policy instruments on renewable energy consumption (REC) is crucial for the design and choice of policy packages. This study aims to empirically investigate the effect of environmental policy stringency index (EPS) and its components on REC in 27 OECD countries over the period from 1990 to 2015, and then use the results to identify what the appropriate environmental policy mix should look like. By relying on the two-step system GMM estimator, we provide evidence that increasing environmental policy stringency as a whole promotes renewable energy consumption in these 27 developed economies. Moreover, policymakers are able, through the market- and non-market-based environmental policy instruments, to increase the use of renewable energy. However, not all of these instruments are effective for achieving this goal. The results indicate that R&D subsidies and trading schemes have a positive and significant impact on REC, while taxes, feed-in tariff and emission standards have not a significant effect. Furthermore, R&D subsidies are more effective than trading schemes for stimulating the use of clean energy. These findings proved to be robust across the three alternative panel techniques used.

Keywords: environmental policy stringency, renewable energy consumption, two-step system-GMM estimation, linear dynamic panel data model

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Authors: Aliyu Aminu Baba

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Ethnic entrepreneurship is defined as a set of connections and regular patterns of interaction among people sharing common national background or migration experience. The disadvantage faced by ethnic minority on paid labour induced them to become self-employed. Also, enclaves motivates trading, creativity, innovation are all to provide specific service or products to certain people. These ethnic minorities are African –Caribbean, Indians, Pakistanis, Banghaladashi and Chinese. For policy development ethnic diversity was among the problem of developing policy in United Kingdom. The study finds that there is a danger in treating all ethnic minority businesses as homogeneous rather than heterogeneous. The diversity is due to religious beliefs, culture and race. This indicates that there is a wide range have shortfall in addressing the peculiarities of ethnic minority businesses in policy formulation. Also, there are differences between ethnic minorities in accessing finance. It is recommended that diversity and peculiarities between ethnic minorities should be considered in policy formulation.

Keywords: ethnic entrepreneurship, finance, policy implication, diversity

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Authors: Babatunde Osabiya

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Cybersecurity has emerged as a pressing policy problem in recent years, affecting individuals, businesses, and governments worldwide. This research paper aims to critically review the literature on cybersecurity policy and apply policy theory to propose a policy approach that balances the freedom to access and use technology with the human rights risks and threats posed by cyber. Drawing on various credible sources, the paper examines the scale and seriousness of cyber threats, highlighting the growing threat posed by cybercriminals, hackers, and nation-states. The paper also identifies the key challenges facing policymakers, including the need for more significant investment in cybersecurity research and development and the importance of balancing the benefits of technological innovation with the risks to privacy, security, and human rights. To address these challenges, the paper proposes a policy approach emphasizing investing in cybersecurity research and development to maintain a technological edge over potential adversaries. This approach also highlights the need for greater collaboration between government, industry, and civil society to develop effective cybersecurity policies and practices that protect the rights and freedoms of people while mitigating the risks posed by cyber threats. This paper will contribute to the growing body of literature on cybersecurity policy and offers a policy framework for addressing this critical policy challenge.

Keywords: security risk, legal framework, cyber security and policy, national security

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Authors: Anshul Raja

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Cystic lesions of the spleen are rare and splenic hydatid cysts account for only 0.5% to 8% of all hydatidosis. Authors hereby report a case where a 50-year-old female presented to our hospital with the complains of heaviness and pain over left upper abdomen over the past 8-10 years. On radiological examination, ultrasonography revealed findings consistent with isolated splenic hydatid cyst and was later on confirmed on Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). No other organ or system involvement was seen. The patient underwent splenectomy and hydatid cyst was confirmed on histopathology. Owing to its rarity, it offers a diagnostic challenge to physicians but can reliably be diagnosed with great confidence employing various imaging modalities like CT and MRI.

Keywords: gastrointestinal radiology, abdominal imaging, hydatid cyst, medical and health sciences

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Authors: Julia Kapr, Andrea Rossi, Haribaskar Ramachandran, Marius Pollet, Ilka Egger, Selina Dangeleit, Katharina Koch, Jean Krutmann, Ellen Fritsche

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It is widely acknowledged that animal models do not always represent human disease. Especially human brain development is difficult to model in animals due to a variety of structural and functional species-specificities. This causes significant discrepancies between predicted and apparent drug efficacies in clinical trials and their subsequent failure. Emerging alternatives based on 3D in vitro approaches, such as human brain spheres or organoids, may in the future reduce and ultimately replace animal models. Here, we present a human induced pluripotent stem cell (hiPSC)-based 3D neural in a vitro disease model for the Cockayne Syndrome B (CSB). CSB is a rare hereditary disease and is accompanied by severe neurologic defects, such as microcephaly, ataxia and intellectual disability, with currently no treatment options. Therefore, the aim of this study is to investigate the molecular and cellular defects found in neural hiPSC-derived CSB models. Understanding the underlying pathology of CSB enables the development of treatment options. The two CSB models used in this study comprise a patient-derived hiPSC line and its isogenic control as well as a CSB-deficient cell line based on a healthy hiPSC line (IMR90-4) background thereby excluding genetic background-related effects. Neurally induced and differentiated brain sphere cultures were characterized via RNA Sequencing, western blot (WB), immunocytochemistry (ICC) and multielectrode arrays (MEAs). CSB-deficiency leads to an altered gene expression of markers for autophagy, focal adhesion and neural network formation. Cell migration was significantly reduced and electrical activity was significantly increased in the disease cell lines. These data hint that the cellular pathologies is possibly underlying CSB. By induction of autophagy, the migration phenotype could be partially rescued, suggesting a crucial role of disturbed autophagy in defective neural migration of the disease lines. Altered autophagy may also lead to inefficient mitophagy. Accordingly, disease cell lines were shown to have a lower mitochondrial base activity and a higher susceptibility to mitochondrial stress induced by rotenone. Since mitochondria play an important role in neurotransmitter cycling, we suggest that defective mitochondria may lead to altered electrical activity in the disease cell lines. Failure to clear the defective mitochondria by mitophagy and thus missing initiation cues for new mitochondrial production could potentiate this problem. With our data, we aim at establishing a disease adverse outcome pathway (AOP), thereby adding to the in-depth understanding of this multi-faced disorder and subsequently contributing to alternative drug development.

Keywords: autophagy, disease modeling, in vitro, pluripotent stem cells

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Authors: Antarip Bhattacharya, Dhritiman Maitra

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Primary hyperparathyroidism (PHPT) is the most common cause of hypercalcemia due to autonomous production of parathormone (PTH) and the third most common endocrine disorder. Upto 2% of postmenopausal women could have this condition. Primary hyperparathyroidism is characterized by hypercalcemia with a high or insufficiently suppressed level of parathyroid hormone and is caused by a solitary parathyroid adenoma in 85-90% of patients. PHPT may also be caused by parathyroid hyperplasia (involving multiple glands) or parathyroid carcinoma. Associated morbidities and sequelae include decreased bone mineral density, fractures, kidney stones, hypertension, cardiac comorbidities and psychiatric disorder which entail huge costs for treatment. In the year 2021, by virtue of running a Breast and Endocrine Surgery clinic in a Tier 1 city at a tertiary care hospital, the opportunity to be associated with patients of hyperparathyroidism came our way. Here, we shall describe the spectrum of clinical presentations and customisation of treatment for parathyroid diseases with reference to the above patients. A retrospective analysis of the data of all patients presenting with symptoms of parathyroid diseases was made and classified according to the cause. 13 patients had presented with symptoms of hyperparathyroidism and each case presented with unique symptoms and necessitated detailed evaluation. The treatment or surgery offered to each patient was tailored to his/her individual disease and led to favourable outcomes. Diseases affecting parathyroid are not as rare as we believe. Each case merits detailed clinical evaluation, investigations and tailoring of suitable treatment with regard to medical management and extent of surgery. Intra-operative frozen section/iOPTH monitoring are really useful adjuncts for intra-operative decision making.

Keywords: hyperparathyroidism, parathyroid adenoma, parathyroid surgery, PTH

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Authors: Shahla Afshar Paiman, Sedigheh Madani, Zahra Hosseininezhad

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Introduction: The most common causes of appendix luminal obstruction are fecaliths and lymphoid follicle hyperplasia. Appendicitis is a very rare Gastrointestinal complication of varicella zosterand it is mostly observed in immune-compromised patient. Case presentation: Here we reported a case of varicella zoster-related perforated appendicitis with synchronous encephalopathy as a first presentation of chickenpox in a 10-year-old boy. He had no history of immunodeficiency or predisposing factors and his diagnosis is confirmed by both serological lab tests and abdominal fluid (peritoneal secretion) PCR. Conclusion: Varicella zoster could cause appendicitis as first presentation, along with other critical complications look likes encephalopathy.

Keywords: Varicella zoster, appendicitis, encephalitis, children

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Authors: Makda Aamir, Sood Aayushi, Syed Omar, Nihan Khuld, Iskander Peter, Ijaz Naeem, Sharma Nishant

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Introduction:Glycogen Storage Disease Type-1 (GSD-1) is a rare phenomenon primarily affecting the liver and kidney. Excessive accumulation of glycogen and fat in liver, kidney, and intestinal mucosa is noted in patients with deficiency of Glucose-6-phosphatase deficiency. Patients with GSD-1 have a wide spectrum of symptoms, including hepatomegaly, hypoglycemia, lactic acidemia, hyperlipidemia, hyperuricemia, and growth retardation. Age of onset, rate of disease progression and its severity is variable in this disease.Case:An 18-year-old male with GSD-1a, Von Gierke’s disease, hyperuricemia, and hypertension presented to the hospital with nausea and vomiting. The patient followed an hourly cornstarch regimen during the day and overnight through infusion via a PEG tube. The complaints started at work, where he was unable to tolerate oral cornstarch. He washemodynamically stable on arrival. ABG showed pH 7.372, PaCO2 30.3, and PaO2 92.2. WBC 16.80, K+ 5.8, HCO3 13, BUN 28, Cr 2.2, Glucose 60, AST 115, ALT 128, Cholesterol 352, Triglycerides >1000, Uric Acid 10.6, Lactic Acid 11.8 which trended down to 8.0. CT abdomen showed hepatomegaly and fatty infiltration with the PEG tube in place.He was admitted to the ICU and started on D5NS for hypoglycemia and lactic acidosis. Per request by the patient’s pediatrician, he was transitioned to IV D10/0.45NS at 110mL/Hr to maintain blood glucose above 75 mg/L. Frequent accuchecks were done till he could tolerate his dietary regimen with cornstarch. Lactic acid downtrend to 2.9, and accuchecks ranged between 100-110. Cr improved to 1.3, and his home medications (Allopurinol and Lisinopril) were resumed. He was discharged in stable condition with plans for further genetic therapy work up.Discussion:Mainstay therapy for Von Gierke’s Disease is the prevention of metabolic derangements for which dietary and lifestyle changes are recommended. A low fructose and sucrose diet is recommended by limiting the intake of galactose and lactose to one serving per day. Hypoglycemia treatment in such patients is two-fold, utilizing both quick and stable release sources. Cornstarch has been one such therapy since the 1980s; its slow digestion provides a steady release of glucose over a longer period of time as compared with other sources of carbohydrates. Dosing guidelines vary from age to age and person to person, but it is highly recommended to check BG levels frequently to maintain a BG > 70 mg/dL. Associated high levels of triglycerides and cholesterol can be treated with statins, fibrates, etc. Conclusion:The management of hypoglycemia in GSD 1 disease presents various obstacles which could prove to be fatal. Due to the deficiency of G6P, treatment with a specialized hypoglycemic regimen is warranted. A D10 ½ NS infusion can be used to maintain blood sugar levels as well as correct metabolic or lactate imbalances. Infusion should be gradually weaned off after the patient can tolerate oral feeds as this can help prevent the risk of hypoglycemia and other derangements. Further research is needed in regards to these patients for more sustainable regimens.

Keywords: von gierke, glycogen storage disease, hypoglycemia, genetic disease

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Authors: Angeliki Vassila, Dimitrios Kalogeropoulos, Rania Rawashdeh, Nigel Hall, Najiha Rahman, Mark Fabian, Suresh Thulasidharan, Hossain Parwez

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Introduction: Necrotising scleritis is a severe form of scleritis and poses a significant threat to vision. It can manifest in various systemic autoimmune disorders, systemic vasculitis, or as a consequence of microbial infections. The objective of this study is to present a case of necrotizing scleritis associated with scleroderma, which was further complicated by a secondary Staphylococcus epidermidis infection. Methods: This is a retrospective analysis that examines the medical records of a patient who was hospitalised in the Eye Unit at University Hospital Southampton. Results: A 78-year-old woman presented at the eye casualty department of our unit with a two-week history of progressively worsening pain in her left eye. She received a diagnosis of necrotising scleritis and was admitted to the hospital for further treatment. It was decided to commence a three-day course of intravenous methylprednisolone followed by a tapering regimen of oral steroids. Additionally, a conjunctival swab was taken, and two days later, it revealed the presence of S. epidermidis, indicating a potential secondary infection. Given this finding, she was also prescribed topical (Ofloxacin 0.3% - four times daily) and oral (Ciprofloxacin 750mg – twice daily) antibiotics. The inflammation and symptoms gradually improved, leading to the patient being scheduled for a scleral graft and applying an amniotic membrane to cover the area of scleral thinning. Conclusions: Rheumatoid arthritis and granulomatosis with polyangiitis are the most commonly identifiable systemic diseases associated with necrotising scleritis. Although association with scleroderma is extremely rare, early identification and treatment are necessary to prevent scleritis-related complications.

Keywords: scleritis, necrotizing scleritis, scleroderma, autoimmune disease

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Authors: Markéta Slováková

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The largest share of policy and money within the European Union goes to agriculture. The Union’s Common Agricultural Policy has undergone several transformations in the last five decades, with the main change taking place in the 1990's. This change influenced agriculture in the Czech Republic, inasmuch as the fledgling republic was preparing to join the European Union and adopt its policies. In the 1990s, Czech agriculture passed from a centrally planned economy to a market economy and subsequently adopted the terms of the Common Agricultural Policy. The Czech Republic is also characterized by a significant landscape sphere diversification. Agricultural entrepreneurs in the Czech Republic are still not used the possibility of grants from the European Union. They focus rather on national or regional subsidy titles. Only half of all agricultural entrepreneurs in the Czech Republic use European subsidies. This article focuses on the introduction of the Common Agricultural Policy to the Czech Republic and its subsequent influence on Czech agriculture. It is demonstrated on the implementation rate of the CAP in the EU Member States and the closer focus is on the Czech integration.

Keywords: common agricultural policy, agriculture, European Union, transformation

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Authors: Anum Akhter, Sumaira Altaf

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Purpose: To study the effect of low vision devices i.e. telescope and magnifying glasses on distance visual acuity and near visual acuity of children with Stargardt’s disease. Setting: Low vision department, Alshifa Trust Eye Hospital, Rawalpindi, Pakistan. Methods: 52 children having Stargardt’s disease were included in the study. All children were diagnosed by pediatrics ophthalmologists. Comprehensive low vision assessment was done by me in Low vision clinic. Visual acuity was measured using ETDRS chart. Refraction and other supplementary tests were performed. Children with Stargardt’s disease were provided with different telescopes and magnifying glasses for improving far vision and near vision. Results: Out of 52 children, 17 children were males and 35 children were females. Distance visual acuity and near visual acuity improved significantly with low vision aid trial. All children showed visual acuity better than 6/19 with a telescope of higher magnification. Improvement in near visual acuity was also significant with magnifying glasses trial. Conclusions: Low vision aids are useful for improvement in visual acuity in children. Children with Stargardt’s disease who are having a problem in education and daily life activities can get help from low vision aids.

Keywords: Stargardt, s disease, low vision aids, telescope, magnifiers

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Authors: Lillie Lum

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Focusing on the role of official language in immigrant integration, this paper will first report the results of a literature review and demonstrate that there is no doubt on the necessity of adequate language skills for newcomers to successfully settle, adapt, and integrate socially, culturally and economically in Canada. This paper attempts to synthesize the literature in order to shed light on the language policy terrain which is not easy to navigate. Then, by outlining what is currently available in the language policy environment, it will ask if the current state of language training in Canada is adequate to assist newcomers in their language acquisition process. At a deeper level, it aims to continue to raise questions in this policy area. Are current policy responses likely to improve linguistic capabilities in the future, particularly for immigrant workers with poor language proficiency? This paper is timely given the magnitude of the language issue and the value of immigrants for Canada’s economic, social, and political vitality.

Keywords: official language education, immigrant integration into Canada, economic factors, policy implications

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Authors: Yousef Alwashahi, Ahmed Almoqbali, Mayar Albahrani, Asma Alajmi

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We report a rare case of a 49-year-old Omani woman who is a known case of primary anti-phospholipid syndrome, glucose-6-phosphate dehydrogenase deficiency, and iron deficiency anaemia. During cannulation, she was found to develop bulla that progressed to ulcerations. With chronicity and recurrent abscess formation that usually increase after surgical intervention, a pathergy phenomenon was postulated. High suspicion of pyoderma gangrenosum was considered. Fortunately, the rapid progression of the disease was slowed down with corticosteroids, cyclosporin, and biological agents.

Keywords: anti-phospholipid syndrome, pyoderma gangrenosum, bullous pyoderma gangrenosum, pathergy, pathergy phenomenon

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Authors: Thieu-Thi Tra My

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Undifferentiated embryonal sarcoma of the liver (UESL), a rare malignant mesenchymal tumor, is commonly seen in children. The symptoms and imaging were not specific, so it could be mimicked with other tumors or liver abscesses. The tumor often appears as a large heterogeneous echoic solid mass with small cystic areas while showing a cyst-like appearance on CT and MRI. The histopathological manifestation of the UESL consisted of stellate-shaped and spindle cells scattered on a myxoid background with high mitotic count. Cells with multiple or bizarre nuclear were also observed. Here, we aimed to describe a 9-year-old male diagnosed with UESL focused on imaging and histopathological characteristics.

Keywords: undifferentiated embryonal sarcoma of liver, UESL, liver sarcoma, liver tumor, children

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Authors: K. Assis, K. P. Chong, A. S. Idris, C. M. Ho

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Oil palm or Elaeis guineensis is considered as the golden crop in Malaysia. But oil palm industry in this country is now facing with the most devastating disease called as Ganoderma Basal Stem Rot disease. The objective of this paper is to analyze the economic loss due to this disease. There were three commercial oil palm sites selected for collecting the required data for economic analysis. Yield parameter used to measure the loss was the total weight of fresh fruit bunch in six months. The predictors include disease severity, change in disease severity, number of infected neighbor palms, age of palm, planting generation, topography, and first order interaction variables. The estimation model of yield loss was identified by using backward elimination based regression method. Diagnostic checking was conducted on the residual of the best yield loss model. The value of mean absolute percentage error (MAPE) was used to measure the forecast performance of the model. The best yield loss model was then used to estimate the economic loss by using the current monthly price of fresh fruit bunch at mill gate.

Keywords: ganoderma, oil palm, regression model, yield loss, economic loss

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Authors: Temosho Promise Chuene, T. Chitura

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Background: Foot and mouth disease is a real challenge in South Africa. The disease is a serious threat to the viability of livestock farming initiatives and affects local and international livestock trade. In Limpopo Province, the Kruger National Park and other game reserves are home to the African buffalo (Syncerus caffer), a notorious reservoir of the picornavirus, which causes foot and mouth disease. Out of the virus’s seven (7) distinct serotypes, Southern African Territories (SAT) 1, 2, and 3 are commonly endemic in South Africa. The broad objective of the study was to establish the trend of foot and mouth disease in Limpopo Province over a seven-year period (2014-2020), as well as the adoption and comprehensive reporting of the measures that are taken to contain disease outbreaks in the study area. Methods: The study used secondary data from the World Organization for Animal Health (WOAH) on reported cases of foot and mouth disease in South Africa. Descriptive analysis (frequencies and percentages) and Analysis of variance (ANOVA) were used to present and analyse the data. Result: The year 2020 had the highest prevalence of foot and mouth disease (3.72%), while 2016 had the lowest prevalence (0.05%). Serotype SAT 2 was the most endemic, followed by SAT 1. Findings from the study demonstrated the seasonal nature of foot and mouth disease in the study area, as most disease cases were reported in the summer seasons. Slaughter of diseased and at-risk animals was the only documented disease control strategy, and information was missing for some of the years. Conclusion: The study identified serious underreporting of the adopted control strategies following disease outbreaks. Adoption of comprehensive disease control strategies coupled with thorough reporting can help to reduce outbreaks of foot and mouth disease and prevent losses to the livestock farming sector of South Africa and Limpopo Province in particular.

Keywords: livestock farming, African buffalo, prevalence, serotype, slaughter

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Authors: Philipp Galkin

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Government policy is a critical factor in the understanding of energy markets. Regardless, it is rarely approached systematically from a research perspective. Gaining a precise understanding of what policies exist, their intended outcomes, geographical extent, duration, evolution, etc. would enable the research community to answer a variety of questions that, for now, are either oversimplified or ignored. Policy, on its surface, also seems a rather unstructured and qualitative undertaking. There may be quantitative components, but incorporating the concept of policy analysis into quantitative analysis remains a challenge. The KAPSARC Energy Policy Database (KEPD) is intended to address these two energy policy research limitations. Our approach is to represent policies within a quantitative library of the specific policy measures contained within a set of legal documents. Each of these measures is recorded into the database as a single entry characterized by a set of qualitative and quantitative attributes. Initially, we have focused on the major laws at the national level that regulate coal in China. However, KAPSARC is engaged in various efforts to apply this methodology to other energy policy domains. To ensure scalability and sustainability of our project, we are exploring semantic processing using automated computer algorithms. Automated coding can provide a more convenient input data for human coders and serve as a quality control option. Our initial findings suggest that the methodology utilized in KEPD could be applied to any set of energy policies. It also provides a convenient tool to facilitate understanding in the energy policy realm enabling the researcher to quickly identify, summarize, and digest policy documents and specific policy measures. The KEPD captures a wide range of information about each individual policy contained within a single policy document. This enables a variety of analyses, such as structural comparison of policy documents, tracing policy evolution, stakeholder analysis, and exploring interdependencies of policies and their attributes with exogenous datasets using statistical tools. The usability and broad range of research implications suggest a need for the continued expansion of the KEPD to encompass a larger scope of policy documents across geographies and energy sectors.

Keywords: China, energy policy, policy analysis, policy database

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