jimnoneill/paper-to-field-training huggingface dataset:
Clinicopathological spectrum and recent advances in the treatment of hereditary transthyretin amyloidosis

Abstract Hereditary transthyretin (ATTRv) amyloidosis, also known as familial amyloid polyneuropathy, is a disease caused by the systemic deposition of variant transthyretin (TTR). Although this disease was primarily indigenous to endemic foci in Portugal, Japan, and Sweden, its prevalence has increased throughout the world. The clinical phenotypes of ATTRv amyloidosis are diverse, including neuropathy, cardiomyopathy, and oculoleptomeningeal involvement as the predominant features, depending on the mutation type and age of onset. Val30Met, one of the most common TTR mutations, exhibits varying characteristic features in the early‐onset patients from conventional endemic foci and the late‐onset patients from non‐endemic areas. Autonomic dysfunctions and dissociated sensory loss are the characteristic features of the former, whereas motor dysfunctions and loss of all sensory modalities are conspicuous in the latter. Distortion and atrophy of the Schwann cells due to the formation of amyloid fibrils seem to cause predominant small‐fiber loss in the early‐onset patients, while other mechanisms, such as microangiopathy and the toxicity of TTR oligomers, may contribute to nerve fiber loss in the late‐onset patients. In addition to liver transplantation, novel and less invasive disease‐modifying therapies, such as TTR stabilizers, short interfering RNA, and antisense oligonucleotide, have been shown to be effective in ATTRv amyloidosis patients. Given their ability to ameliorate ATTRv amyloidosis, early diagnosis and treatment are required. Physicians should be aware of the diversity in ATTRv amyloidosis because this disease can be misdiagnosed with other diseases such as chronic inflammatory demyelinating polyneuropathy and immunoglobulin light‐chain amyloidosis.

Topic: Amyloidosis: Diagnosis, Treatment, Outcomes
Subfield: Molecular Biology
Field: Biochemistry, Genetics and Molecular Biology
Domain: Life Sciences
Pituitary stalk interruption syndrome: case report of a rare disease requiring prompt detection

Pituitary stalk interruption syndrome (PSIS) is a rare congenital abnormality of the pituitary gland, which consists of the triad of thin or interrupted pituitary stalk, small or absent anterior pituitary gland, and an absent or ectopic posterior pituitary lobe. Clinical presentation depends on the age at the time of diagnosis and PSIS should always be suspected in children with delayed puberty, short stature, and/or anterior pituitary deficiency. Reported incidence of PSIS is increasing because of the growing use in clinical practice of Magnetic Resonance (MR) as a primary radiological modality in patients with hypopituitarism, and MR findings are pathognomonic of this disease. Neuroradiological investigations may also reveal other abnormalities, as PSIS can be associated with various extra-cerebral midline defects. Without early diagnosis and treatment, mortality and morbidity in these patients is high. On the other hand, early treatment of this rare disease can prevent complications and permanent short statue of the patients. In this paper, we presented a classic case of PSIS in an 11-year-old male, with emphasis to imaging findings and differential diagnosis of this rare syndrome, which requires prompt detection to gain the maximum therapeutic benefit.

Topic: Pituitary Gland Disorders and Treatments
Subfield: Endocrinology, Diabetes and Metabolism
Field: Medicine
Domain: Health Sciences
Attitude and Orbit Control Result of Super Low Altitude Test Satellite “TSUBAME'' (SLATS)

Super Low Altitude Test Satellite (SLATS) nicknamed "TSUBAME'' was successfully launched on December 23, 2017. The purposes of SLATS are 1) Test of keeping satellite's altitude with its own ion engine against high atmospheric drag at super low altitude, 2) Data acquisition of atmospheric density and atomic oxygen, and 3) Test of optical earth observations. SLATS operation was finished on October 1, 2019. SLATS got following results in terms of the attitude and orbit control. 1) Orbit transfer from initial orbit which is 643 Χ 450km elliptic and descending node local sun time (LST) 10:30 orbit to initial super low altitude orbit which is 271.5km circle and LST 16:00 orbit using a chemical gas jet system (RCS) and an atmospheric drag for about one year, 2) Altitude keeping at 271.5, 216.8km altitude for 38days respectively and at 250, 240, 230, 181.1, 167.4km altitude for 7days respectively, 3) Guidance and longitude keeping at 271.5km/1day, 216.8km/5days and 181.1km/3 days recurrent orbits. This paper shows the on-orbit results in terms of the attitude and orbit control of SLATS.

Topic: Inertial Sensor and Navigation
Subfield: Aerospace Engineering
Field: Engineering
Domain: Physical Sciences
An Analysis of the Relationship Between Work Satisfaction and Organizational Commitment

This article investigates the interrelationship between individual characteristics, work rewards, work satisfaction, and organizational commitment. Data obtained from 1,385 workers representing a variety of occupational groups are analyzed. Generally, the data suggest that individual characteristics have very little impact on either satisfaction or commitment, while work rewards are found to be better predictors of satisfaction than commitment. More important, the data further indicate that satisfaction and commitment have reciprocal effects; however, it appears that satisfaction has a significantly greater effect on commitment than the reverse. The implications of these findings are discussed.

Topic: Job Satisfaction and Organizational Behavior
Subfield: Organizational Behavior and Human Resource Management
Field: Business, Management and Accounting
Domain: Social Sciences
Fostering Food Market Development in Zambia

This article assesses major features of the Zambian agricultural market that have led to the underdevelopment of the staple food marketing system, reviews existing empirical evidence explaining the variable performance of the maize sub-sector, and suggests potential corrective measures. As smallholder farmers in agricultural markets are considered to be vitally important to the attainment of food security, special attention is placed on how the current system affects these farmers, and on how to foster improved participation. The role and potential of new generation cooperatives is also considered.We identify seven major areas in need of serious and coordinated attention if the state of the agricultural sector and marketing functions is to improve.

Topic: Innovation and Socioeconomic Development
Subfield: Business and International Management
Field: Business, Management and Accounting
Domain: Social Sciences
DOP64 Pathogenic RIPK1 Mutations Cause Infantile-onset IBD with Inflammatory and Fistulizing Features

Abstract Background Genetics plays a key role in the pathogenesis of inflammatory bowel disease (IBD). With the expanding use of next-generation sequencing, &amp;gt;70 different monogenic disorders associated with IBD have been identified, and most of them present in the first years of life. Recently, several patients with severe IBD were identified to harbor pathogenic mutations in Receptor-interacting serine/threonine-protein kinase 1 (RIPK1) gene, which regulates necroptosis, a necrotic cell death mechanism. We present the clinical features, genetic analysis and immune work-up of three patients with infantile-onset IBD resulting from novel RIPK1 mutations. Methods Whole exome sequencing was performed in three patients with severe infantile-onset IBD, along with sanger sequencing for conformation. Mass cytometry time of flight was conducted for in-depth immunophenotyping, including cytokine secretion analysis following lipopolysaccharide (LPS) or Phorbol myristate acetate with ionomycin (PMA-I), on one of the patient’s peripheral blood mononuclear cells, and compared to control subjects and patients with Crohn’s disease. Results All patients, born to consanguineous Muslim families, presented with severe colitis and multiple perianal fistulas in the first months of life, without severe or atypical infections. One of the patients had a partial response to high doses of inlfliximab and azathioprine, while another one failed to respond to adaliumab and later to low dose anakinra, an IL-1 receptor antagonist. Genetic studies identified novel and pathogenic genetic variants in the RIPK1 gene in all patients, that were confirmed by Sanger sequencing. Using mass cytometry time of flight unbiased clustering analysis, we identified peripheral immune dysregulation in one of these patients, characterized by an increase in IFNγ CD8+ T cells along with a decrease in monocytes, dendritic cells and B cells. Moreover, RIPK1-deficient patient’s immune cells exhibited decreased IL-6 production in response to LPS across multiple cell types including T cells B cells and innate immune cells. Conclusion Mutations in RIPK1 should be considered in very young patients presenting with colitis and perianal fistulas. Given RIPK1’s role in both immune cells (and specifically in inflammasome activation), and epithelial cells, it is unclear whether immunosuppressive medications (including IL1 blockade) as well as allogeneic hematopoietic stem cell transplantation can suppress or cure the hyper-inflammatory response in these patients. Additional studies in humans are required to better define the role of RIPK1 in regulating intestinal immune responses, and how treatment can be optimized for patients with RIPK1 deficiency.

Topic: Immunodeficiency and Autoimmune Disorders
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
The Influence of Summertime Heat Waves on Emotional Health

Abstract Background: With increases in global urbanization and global warming, there are corresponding increases in urban heat island effects. Heat wave disasters are occurring more frequently, posing direct and indirect mental health hazards to urban residents. The impact of high ambient temperatures on emotional health is a scientific problem that needs clarification. Methods: Data collected through 386 valid emotions questionnaire and temperature data measured on heat wave days were analyzed using GIS, SPSS and MATLAB software to study the influence of heat waves on the negative emotions of middle-aged and older adults (over 40-years-old). Results: The results indicate that the degree of influence of high temperatures on various negative emotions differs significantly, as some emotional responses tend to fluctuate while others steadily worsen. The progression of emotional responses is distressed &gt; irritable &gt; nervous &gt; hostile. With increasing temperatures, phase-based changes in emotional states occur, with different emotional states corresponding to different temperature thresholds; the temperature thresholds for distress, nervousness, and hostility were shown to be 38°C/43°C, 40°C/44°C, 43°C, respectively. Conclusions: Ambient temperatures exceeding 35°C have a significant negative impact on the emotional health of middle-aged and older adults. Irritability and nervousness monotonically increase, while distress and hostility tend to fluctuate. This study contributes to the prevention and management of the harmful effects of heat waves on emotions, providing basic information applicable to the design of layouts for urban green spaces to reduce the effects of urban heat islands.

Topic: Climate Change and Health Impacts
Subfield: Health, Toxicology and Mutagenesis
Field: Environmental Science
Domain: Physical Sciences
THE NATIONAL COORDINATION OF EU POLICY IN HUNGARY: PATTERNS OF CONTINUITY AND CHANGE

Although European Union (EU) membership generates similar functional pressures for national administrations, member states developed institutional arrangements that show marked cross‐country variation in the coordination of EU policy. This article examines and assesses the institutions and mechanisms established in Hungary for formulating policy positions on the domestic level. In line with the general features of central government, the system is highly centralized and hierarchical, and has been characterized by a comprehensive coordination ambition from its inception. The case confirms the primary importance of national institutional factors for shaping coordination systems, but also the relevance of more actor‐centered explanations for accounting for changes in the top decision‐making tier. At the same time, the Hungarian experience also draws attention to power relations within governing parties and prime ministerial involvement as important variables so far relatively neglected in the literature.

Topic: European Union Policy and Governance
Subfield: Political Science and International Relations
Field: Social Sciences
Domain: Social Sciences
Counterfactual Story Reasoning and Generation

Lianhui Qin, Antoine Bosselut, Ari Holtzman, Chandra Bhagavatula, Elizabeth Clark, Yejin Choi. Proceedings of the 2019 Conference on Empirical Methods in Natural Language Processing and the 9th International Joint Conference on Natural Language Processing (EMNLP-IJCNLP). 2019.

Topic: Topic Modeling
Subfield: Artificial Intelligence
Field: Computer Science
Domain: Physical Sciences
Robust estimation of the total unit hydrograph

This paper investigates three techniques for estimating the parameter values for the unit hydrograph: full model optimisation (the CWI and CMD versions of IHACRES were used), simple rainfall scaling approaches for estimating effective rainfall and an inverse filtering approach. The approaches are tested using synthetic streamow data generated by the catchment moisture deficit (CMD) version of the IHACRES model, based on observed rainfall data for Cotter catchment (near Canberra, Australia) and the Singkarak catchment (Sumatra, Indonesia). The impact of rainfall and streamow errors are considered by reducing the raingauge density and introducting errors in the rating curve. The inverse filtering apprach performed well for the synthetic streamow derived from the rainfall data for the Cotter catchment. For the Singkarak catchment data, the differences between the approaches was small, due possibly to either the higher frequency of rainfall in that area, or to the lower correlation between the rainfall gauge locations.

Topic: Hydrology and Watershed Management Studies
Subfield: Water Science and Technology
Field: Environmental Science
Domain: Physical Sciences
Public perceptions of Internet‐based health scams, and factors that promote engagement with them

The prevalence of health scams in Canada is increasing, facilitated by the rise of the Internet as a mass communication medium. However, little is known about the nature of this phenomena. Building on previous work exploring the nature of Internet health scams (IHS), this project sought to better understand the reasons why people engaged with IHS, and if contemporary psychosocial theory can help explain IHS engagement. A mixed-methods study, involving a web-based survey incorporating qualitative questions and the Susceptibility to Persuasion-II Brief psychometric scale (STP-II Brief), were administered (N = 194) in British Columbia, Canada, in 2017. Results (n = 156) demonstrated that 40% of participants had ever engaged with IHS, but only 1% reported to have actually lost money to a deceptive product/service. Associations between scam engagement, participant demographics and STP-II Brief scores were explored, with Sex and Employment Status both found to have a significant effect on odds of IHS engagement. STP-II Brief scores were positively correlated with a likelihood of engagement with IHS, even when adjusting for demographic characteristics. The types of IHS most frequently engaged with were those related to body image products, and social influence appeared to be a dominant psychosocial factor promoting engagement. Participants reported that claims of products being 'natural', the result of scientific breakthroughs, use of pseudoscientific language, use of testimonials, and celebrity or professional endorsement could lead them to engage with a product. These findings can help inform health professionals' understanding of public health-seeking behaviours with respect to deceptive marketing.

Topic: Digital Communication and Language
Subfield: Human-Computer Interaction
Field: Computer Science
Domain: Physical Sciences
Multivariate Standard Addition Cobalt Electrochemistry Data Fusion for Determining Phosphate Concentration in Hydroponic Solution

Phosphate is one of the major elements affecting agricultural production. The accurate determination of phosphate concentration essential for plant growth, especially in a hydroponics system, allows regulating the balanced and suitable range set of nutrients to plants efficiently. This study proposed a data fusion model based on 70 samples for calibration and 30 samples for predicting concentrations of phosphate in an eggplant nutrient solution. Three multivariate analysis methods i.e. partial least squares model (PLS), Gaussian process regression (GPR), and artificial neural network (ANN) were studied and compared for their performance efficiencies. The results showed that combining the multivariate standard addition method (MSAM) in acquiring data from cobalt electrodes and ANN data fusion model came up with satisfactory outcomes. Both the method provided good performance with R<sup>2</sup> values of 0.98 and 0.96, and the root mean square error (RMSE) of 50 and 66 mg. L<sup>-1</sup> respectively in calibration and evaluation tests. These values were much higher than those of conventional processing techniques. Moreover, the normal direct calibration method in acquisition signal from cobalt electrodes was also applied, which provided R<sup>2</sup> values of 0.7 to 0.8. These high values are sufficient for development to measure phosphate concentration in hydroponic solutions.

Topic: Water Quality Monitoring and Analysis
Subfield: Industrial and Manufacturing Engineering
Field: Environmental Science
Domain: Physical Sciences
Syndrome Type II

In autoimmune polyglandular syndromes (APS), several organ-specific autoimmune diseases are clustered. Although APS type I is caused by loss of central tolerance, the etiology of APS type II (APS-II) is currently unknown. However, in several murine models, depletion of CD4 � CD25 � regulatory T cells (T regs) causes a syndrome resembling human APS-II with multiple endocrinopathies. Therefore, we hypothesized that loss of active suppression in the periphery could be a hallmark of this syndrome. T regs from peripheral blood of APS-II, control patients with single autoimmune endocrinopathies, and normal healthy donors showed no differences in quantity (except for patients with isolated autoimmune diseases), in functionally important surface markers, or in apoptosis induced by growth factor withdrawal. Strikingly, APS-II T regs were defective in their suppressive capacity. The defect was persistent and not due to responder cell resistance. These data provide novel insights into the pathogenesis of APS-II and possibly human autoimmunity in general. Key words: suppressor cells • autoimmune polyendocrinopathies • Addison’s disease • type I diabetes • autoimmune thyroiditis

Topic: Adrenal Hormones and Disorders
Subfield: Endocrinology, Diabetes and Metabolism
Field: Medicine
Domain: Health Sciences
Renegotiating the land covenant

ABSTRACT Locating Marlene van Niekerk's novel, Triomf, within a tradition of female responses to the plaasroman, I explore how she uses her novel set on the verge of the 1994 elections to rethink Afrikaners’ relationship with the land. Concentrating on the gendering of relation to the land while attending to intersections of race, gender and class, I analyse her critiques of covenant ideology and mechanised concepts of nature as relations to the land that sanction domination. Invoking Aldo Leopold's concept of a land community, I argue that Van Niekerk points the way to a new land covenant that includes men, women, non-human animals and land as part of an interrelated community. Based on ideals of community, this new land covenant respects difference rather than trying to order it into a hierarchy, and thus it paves the way for a re-imagining of Afrikaner identity for a changing South Africa.

Topic: South African History and Culture
Subfield: Sociology and Political Science
Field: Social Sciences
Domain: Social Sciences
Multidimensional Item Response Theory Models in Vocational Interest Measurement

Vocational interest inventories are commonly analyzed using a unidimensional approach, that is, each subscale is analyzed separately. However, the theories on which these inventories are based often postulate specific relationships between the interest traits. This article presents a multidimensional approach to the analysis of vocational interest data, which takes these relationships into account. Models in the framework of Multidimensional Item Response Theory (MIRT) are explained and applied to a widely used German vocational interest inventory based on the RIASEC model, the AIST-R. MIRT models were more appropriate to describe the data than unidimensional models. It follows that responses to some items were not only influenced by the interest type they were designed to measure but also by another dimension. The advantages of MIRT models are discussed.

Topic: Career Development and Diversity
Subfield: Safety Research
Field: Social Sciences
Domain: Social Sciences
The usefulness of GGE biplot methodology for line × tester data of maize inbred lines

ABSTRACT The genotype + genotype × environment (GGE) biplot methodology has the potential to determine combining ability effects, identify efficient testers, and distinct heterotic groups in a line × tester study. However, it has not been adequately applied for such analysis. Therefore, this study was done to assess the combining ability of some maize inbred lines and testers for grain yield, identify most efficient testers and classify inbred lines into heterotic groups using GGE biplot. Fifteen experimental single cross hybrids, generated from a 5 x 3 line by line × tester crosses procedure, were evaluated across seven sites in Iran in 2018 using a randomized complete block design with three replicates per entry at each site. According to the pooled analysis of variance, crosses showed significant differences for grain yield. Also, the differences observed among the testers, inbred lines, and interactions for the line × tester, crosses × environments, line x tester x environment was significant. The inbred line L4 was recognized as outstanding for its general combining ability (GCA) effect and the tester K18 was highly efficient based on its discriminating power. It can be concluded that the crosses of L4 x K47/3 and L4 x B73 are suggested to release high grain yield hybrids. Results of the GGE biplot analyses were close to the conventional line × tester method in combining ability and heterotic patterns of the yield of lines, grouping, and identification of testers.

Topic: Genetics and Plant Breeding
Subfield: Plant Science
Field: Agricultural and Biological Sciences
Domain: Life Sciences