Miracle-dz/UniFAR-FTUs huggingface dataset:
A Distinct Variant of Pseudohypoparathyroidism (PHP) First Characterized Some 41 Years Ago Is Caused by the 3‐kb STX16 Deletion

In 1980, Farfel and colleagues (NEJM, 1980;303:237–42) provided first evidence for two distinct variants of pseudohypoparathyroidism (PHP) that present with hypocalcemia and impaired parathyroid hormone (PTH)‐stimulated urinary cAMP and phosphate excretion, either in the presence or absence of Albright's hereditary osteodystrophy (AHO). An “abnormal allele” and an “unexpressed allele” were considered as underlying defects, predictions that turned out to be correct for both forms of PHP. Patients affected by the first variant (now referred to as PHP1A) were later shown to be carriers of inactivating mutations involving the maternal GNAS exons encoding Gsα. Patients affected by the second variant (now referred to as PHP1B) were shown in the current study to carry a maternal 3‐kb STX16 deletion, the most frequent cause of autosomal dominant PHP1B, which is associated with loss of methylation at GNAS exon A/B that reduces or abolishes maternal Gsα expression. However, the distinct maternal mutations leading to either PHP1A or PHP1B are disease‐causing only because paternal Gsα expression in the proximal renal tubules is silenced, ie, “unexpressed. ” Our findings resolve at the molecular level carefully conducted investigations reported some 41 years ago that had provided first clues for the existence of two distinct PHP variants. © 2021 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.
Rural Voices on Quality of Service Delivery in Community-Based Health Planning and Services in the Wassa-Amenfi West District, Ghana

The study sought to establish respondents’ views on the attitude of health care providers, perceptions of the quality of medical services provided and indications on the future use of CHPS compound in the Wassa-Amenfi West District. The study employed the cross-sectional survey design within the quantitative research paradigm. Questionnaire was administered to one hundred and fifty community members and analysis was done through frequencies and percentages. The study concluded that respondents had positive views on the attitude of health service providers in the CHPS compound. They were also satisfied with the quality of service delivery at the CHPS compound. Respondents were willing to visit and recommend family members and friends to the facility in the future to access healthcare. It was therefore recommended that medicines and health information should be made available at the compound to enhance quality health delivery at the grass root.
Results of three or more Gamma Knife radiosurgery procedures for recurrent trigeminal neuralgia.

OBJECTIVE Gamma Knife radiosurgery (GKRS) is an established surgical option for the treatment of trigeminal neuralgia (TN), particularly for high-risk surgical candidates and those with recurrent pain. However, outcomes after three or more GKRS treatments have rarely been reported. Herein, the authors reviewed outcomes among patients who had undergone three or more GKRS procedures for recurrent TN. METHODS The authors conducted a multicenter retrospective analysis of patients who had undergone at least three GKRS treatments for TN between July 1997 and April 2019 at two different institutions. Clinical characteristics, radiosurgical dosimetry and technique, pain outcomes, and complications were reviewed. Pain outcomes were scored on the Barrow Neurological Institute (BNI) scale, including time to pain relief (BNI score ≤ III) and recurrence (BNI score > III). RESULTS A total of 30 patients were identified, including 16 women and 14 men. Median pain duration prior to the first GKRS treatment was 10 years. Three patients (10%) had multiple sclerosis. Time to pain relief was longer after the third treatment (p = 0.0003), whereas time to pain recurrence was similar across each of the successive treatments (p = 0.842). Complete or partial pain relief was achieved in 93.1% of patients after the third treatment. The maximum pain relief achieved after the third treatment was significantly better among patients with no prior percutaneous procedures (p = 0.0111) and patients with shorter durations of pain before initiation of GKRS therapy (p = 0.0449). New or progressive facial sensory dysfunction occurred in 29% of patients after the third GKRS treatment and was reported as bothersome in 14%. One patient developed facial twitching, while another experienced persistent lacrimation. No statistically significant predictors of adverse effects following the third treatment were found. Over a median of 39 months of follow-up, 77% of patients maintained complete or partial pain relief. Three patients underwent a fourth GKRS treatment, including one who ultimately received five treatments; all of them reported sustained pain relief at the extended follow-up. CONCLUSIONS The authors describe the largest series to date of patients undergoing three or more GKRS treatments for refractory TN. A third treatment may produce outcomes similar to those of the first two treatments in terms of long-term pain relief, recurrence, and adverse effects.
ESG Importance for Long-Term Shareholder Value Creation: Literature vs. Practice

This article aims to detect how ESG adds value to the long-term shareholder value creation and to discover whether businesses are aware of positive ESG effects and, therefore, whether they will become more ESG-conscious. By conducting a qualitative content analysis on the academic literature, this article firstly aims to determine if shareholders’ value is positively affected by corporate ESG awareness. Secondly, to test whether companies are becoming more conscious about the importance of ESG, the mission statements of publicly listed Central and Eastern European (CEE) companies are compared to their decade-old versions. This analysis allows us to conclude on whether companies have shifted their attention to the ESG factors as a part of their purpose of existence and, therefore, for long-term shareholder value creation, which is one of the main goals of the exchange-listed enterprises. The content analysis results show that companies with higher sustainability awareness ensure shareholder value creation via improved financial performance, management quality as well as reduced risk metrics. Additionally, qualitative nonfinancial factors such as reputation, stakeholder trust, employee satisfaction and engagement provide an even more significant effect on the long-term value than the pure financial matters. The theoretical trend is found to be supported by the fact that sustainability practice and consumer-oriented keywords dominate the mission statements of CEE companies, while keywords related to shareholders and profit experienced the most significant decrease from 2012 to 2021. The present research is unique as it looks at how companies tend to become more ESG aware, integrating the sustainability perspective into their mission statements in response to the global sustainability trend.
Rammed Earth with Straw Fibers and Earth Mortar: Mix Design and Mechanical Characteristics Determination

Raw earth is one of the oldest building materials, which is suitable for various uses: from the construction of load-bearing walls to use for plasters and finishes. The presence of straw fibers can give different behavior to this material. The present paper illustrates preliminary sensory and qualitative analyses, and subsequent laboratory tests that allow the characterization of the raw earth material with straw fibers for rammed earth constructions through mechanized compaction and the identification of a compatible earth mortar. The raw material considered in this study is mainly clayey; for this reason, a mix design usable with the pisé (or clay) technique has been developed. Cylindrical samples have been made through a press and subject to unconfined compression and indirect tensile tests. The results of the tests showed consistent tensile and compressive strength values in the context of earth materials. At the same time, a study for the realization of a mortar with the same base soil was carried out considering four mixtures, in order to investigate the best compromise between workability, shrinkage and compressive strengths. The purpose of the study was to investigate the mechanical characteristics of the local material through preliminary and laboratory tests, to classify it according to the Unified Soil Classification System (USCS) and to verify its suitability for a possible use in the construction field.
Are Parents and Teachers Working Together to Enhance Student Learning?

Ideally both parent and teacher work together for student’ success. The current phenomenon is that many parents with higher education work outside the home with long working hours, so that quantity assistance to children in terms of learning is less, although there are also parents who can maintain the quality of children's learning assistance by facilitating various ways adequate learning or bringing home tutors or facilitating children with online learning. This research aims to examine the relationship between parent and teacher support for students’ success. Thus it can add references for research on the theme of Parents and Teacher Support. A SEM model was used to investigate the correlations among variables via Mplus 8.4. The results showed that the model was fit with RMSEA = . 049; CFI and TLI was . 90, respectively. Parents and Teacher supports were highly correlated significantly (r = .71, p < .05). The implication of these results will be discussed detail at the end of this paper. Keywords—parent support, teacher support, minimum competency assessment, structural equation modelling
‘Cambiando el chip’: The gendered constellation of subjectivities of the financialisation of remittances in Mexico

In the wake of the global financial crisis and a context of stagnating development aid, the international community now promotes linking remittances to finance as a development strategy, in what has been termed the ‘financialisation of remittances’ (FOR). This article analyses the ways in which the financialisation of remittance manifests in Mexico in gendered ways, and what this tells us about financialisation and financial subjectivation processes beyond the global North. We find that the financialisation of remittance represents a shift from earlier remittance-based development models whereby remittances become linked to financial inclusion and social welfare agendas and the focus is broadened beyond migrant income to diaspora wealth. Focusing on the governing arrangements of the financialisation of remittance, we propose the concept of ‘constellation of subjectivities’ in order to analyse the interrelated and interacting programmatic subjectivities through which the financialisation of remittance manifests in Mexico. Combining this conceptualisation with interdisciplinary feminist insights on financialisation, we analyse the various intersecting social dynamics that weave through such constellations. The analysis – based on document, interview and observation material – finds that the financialisation of remittance in Mexico creates and governs a gendered constellation of financial subjectivities with three dimensions: migrant men, remittance-receiving women and the constitutive outside of the non-transnational family. While most studies tend to focus on transnational families, we demonstrate that non-transnational families are an integral part of the financialisation of remittance. Our analysis destabilises the notion of the universal financial subject and highlights the importance of broadening our analysis of financialisation to constitutive outsides that often fall off the radar.
Transformative Service Initiatives: Enabling Access and Overcoming Barriers for People Experiencing Vulnerability

Transformative service initiatives (TSIs) refer to activities by organizations (public, private, nonprofit) or volunteers to serve people experiencing vulnerabilities, including long-term challenges (e.g., refugees, homeless people, undocumented immigrants, ex-convicts) and try to improve their well-being. To advance the concept of TSI, this study proposes a 3A (Awareness, Alignment, Access) Integration Process Framework that can facilitate empirical tests of whether participation in TSIs affects people’s access to critical services. The empirical evidence affirms a positive influence of TSI participation on two TSR outcomes (application and access to higher education), according to data from a longitudinal study of 2,068 refugees (Study 1) that relies on propensity score matching and regression analysis. Study 2 uses qualitative data from six focus groups and identifies 16 barriers to successful refugee integration; it also identifies four individual strategies to overcoming those integration barriers. From academic and public policy perspectives, the findings demonstrate that efforts to plan and implement TSIs should follow a stepwise process to achieve the intended transformative outcomes. These findings can help academic, public policy, and civil society actors design and implement TSIs in various contexts to benefit people experiencing vulnerability.
The Influence of Nation-Level Institutions on Acquisition Premiums: A Cross-Country Comparative Study

We build on neo-institutional theory to examine the manner in which nation-level institutions systematically affect domestic acquisitions—that is, acquisitions involving acquirers and targets from the same country. Specifically, we study in what way premiums are influenced through a set of cognitive, normative, and regulatory forces. In terms of cognitive pressures, we theorize that prior premium decisions of industry peers in the same country influence focal acquisition premiums, since prior premium decisions serve as reference frames for firms. In addition, we posit that normative forces in the form of the national cultural values of uncertainty avoidance, future orientation, and in-group collectivism affect bid premiums, as these factors influence the manner in which firms deal with the uncertainty, payoff time, and merger of groups inherent to acquisitions. Furthermore, we propose that a country’s regulatory pressures through its disclosure requirements influence premiums, since they reduce information asymmetries and affect a firm’s confidence in assessing its potential gains from acquisitions. Using a sample of domestic acquisitions, we find support for several of the hypotheses. Our work offers a cross-country comparative study of how nation-level institutions affect domestic bid premiums and makes theoretical contributions to acquisition premium research and institutional theory.
Exploring the functions of music in the lives of young people on the autism spectrum

Current research investigating the functions of music in everyday life has identified cognitive, emotional, and social functions of music. However, previous research focuses almost exclusively on neurotypical people and rarely considers the musical experiences of autistic people. In addition, there is limited research which focuses explicitly on the musical experiences of young people on the autism spectrum. Current research exploring the functions of music may therefore not accurately represent the experiences of the autistic community. This article aims to explore the function of music in the lives of young people on the autism spectrum through a series of interviews. Eleven young people on the autism spectrum age 12 to 25 (M = 19.4) were interviewed about the function of music in their lives. An adaptive interview technique, utilizing multiple methods of communication, was employed to account for the participants’ broad communicative and personal needs. Interpretative phenomenological analysis revealed four key functions of music in the participants’ lives: Cognitive, Emotional, Social, and Identity. Collectively, these results provide a unique insight into the musical experiences of young people on the autism spectrum.
Impact of Trustworthiness on the Usage of M-banking Apps: A Study on Bangladeshi Consumers

The objective of this study is to find out the impact of trustworthiness on the usage of mobile banking (M-banking) apps in the capital city of Bangladesh. A structured questionnaire was distributed using the snowball sampling technique. Confirmatory factor analyses and structural equation modeling (using SPSS Statistics 20.0 and AMOS 26.0) were used on 362 responses found suitable for the study. The analytical results reveal that all three components of trustworthiness (ability, benevolence, and integrity) have a positive impact on M-banking app usage. Although banks have realized the importance of consumer convenience, they struggle to create trustworthiness. Thus, this study showcases that this positive trustworthiness amongst M-banking app users would be helpful in enhancing the usage of apps.
Enhancing flowering plant functional richness improves wild bee diversity in vineyard inter‐rows in different floral kingdoms

Abstract Wild bees are threatened by multiple interacting stressors, such as habitat loss, land use change, parasites, and pathogens. However, vineyards with vegetated inter‐rows can offer high floral resources within viticultural landscapes and provide foraging and nesting habitats for wild bees. Here, we assess how vineyard management regimes (organic vs. conventional; inter‐row vegetation management) and landscape composition determine the inter‐row plant and wild bee assemblages, as well as how these variables relate to functional traits in 24 Austrian and 10 South African vineyards. Vineyards had either permanent vegetation cover in untilled inter‐rows or temporary vegetation cover in infrequently tilled inter‐rows. Proportion of seminatural habitats (e.g., fallows, grassland, field margins) and woody structures (e.g., woodlots, single trees, tree rows) were used as proxies for landscape composition and mapped within 500‐m radius around the study vineyards. Organic vineyard management increased functional richness (FRic) of wild bees and flowering plants, with woody structures marginally increasing species richness and FRic of wild bees. Wild bee and floral traits were differently associated across the countries. In Austria, several bee traits (e.g., lecty, pollen collection type, proboscis length) were associated with flower color and symmetry, while in South African vineyards, only bees’ proboscis length was positively correlated with floral traits characteristic of Asteraceae flowers (e.g., ray–disk morphology, yellow colors). Solitary bee species in Austria benefitted from infrequent tillage, while ground nesting species preferred inter‐rows with undisturbed soils. Higher proportions of woody structures in surrounding landscapes resulted in less solitary and corbiculate bees in Austria, but more aboveground nesting species in South Africa. In both countries, associations between FRic of wild bees and flowering plants were positive both in organic and in conventional vineyards. We recommend the use of diverse cover crop seed mixtures to enhance plant flowering diversity in inter‐rows, to increase wild bee richness in viticultural landscapes.
A Spatio-Temporal Visualization Approach of PM10 Concentration Data in Metropolitan Lima

Lima is considered one of the cities with the highest air pollution in Latin America. Institutions such as DIGESA, PROTRANSPORTE and SENAMHI are in charge of permanently monitoring air quality; therefore, the air quality visualization system must manage large amounts of data of different concentrations. In this study, a spatio-temporal visualization approach was developed for the exploration of data of the PM10 concentration in Metropolitan Lima, where the spatial behavior, at different time scales, of hourly concentrations of PM10 are analyzed using basic and specialized charts. The results show that the stations located to the east side of the metropolitan area had the highest concentrations, in contrast to the stations located in the center and north that reported better air quality. According to the temporal variation, the station with the highest average of biannual and annual PM10 was the HCH station. The highest PM10 concentrations were registered in 2018, during the summer, highlighting the month of March with daily averages that reached 435 μμg/m3. During the study period, the CRB was the station that recorded the lowest concentrations and the only one that met the Environmental Quality Standard for air quality. The proposed approach exposes a sequence of steps for the elaboration of charts with increasingly specific time periods according to their relevance, and a statistical analysis, such as the dynamic temporal correlation, that allows to obtain a detailed visualization of the spatio-temporal variations of PM10 concentrations. Furthermore, it was concluded that the meteorological variables do not indicate a causal relationship with respect to PM10 levels, but rather that the concentrations of particulate material are related to the urban characteristics of each district.
Delegation in Customs Union under Taste Diversity

ABSTRACT This article investigates how diversity in consumer tastes among potential customs union (CU) members influences delegation decisions between them in the context of CU formation. The model used involves a monopolist, situated in a nonmember country, which chooses the quality of a vertically differentiated good to supply to two potential CU members. Formation of a CU can be sustained only if the monopolist chooses to serve consumers with both low and high maximum willingness to pay in the potential CU members. Delegation decision in setting common external tariff depends on the degree of taste diversity and size of population in each member.
Size-Dependent Properties of Two-Dimensional MoS2 and WS2

The characteristic differences between MoS2 and WS2 nanosheets and nanodots are investigated. The nanosheets were formed by liquid-phase sonication, while the nanodots were formed by breaking the nanosheets through heating the solvent ethylene glycol. The nanosheets and nanodots were approximately 0.7–2 nm thick, with slight deviation. Most of the nanosheets were longer than 100 nm, and most of the nanodots were shorter than 5 nm. As the bulk materials were transformed into nanosheets and/or nanodots, the absorption peaks and Raman peaks shifted to shorter wavelengths. Photoluminescence peaks were observed at 500 and 445 nm in the MoS2 and WS2 samples smaller than 100 nm. In the X-ray diffraction spectra, only the (002) peak was present in the nanosheets, while no peak was detected for the nanodots due to their small size. No detectable differences between the nanosheets and nanodots were observed in the transmission electron micrographs, synchrotron radiation photoemission spectra, or work function measu.
Study on Identification of Several Edible Fungus Strains Using Fourier-transform Infrared Spectroscopy

The spectrograms of Ganoderma lucidum,Stropharia rugoso-annulata and four Lentinula edodes strains were studied using Fourier-transform Infrared Spectroscopy(FTIR). The results indicated that these three kinds of edible fungi could be identified with FTIR because they had specific spectrograms resp.
Studies on Formaldehyde in Lentinus edodes

The 4-amino-3-hydrazino-5-mercapto-1, 2, 4-triazole (AHMT) method was superior to acetylacetone method and chromotropic acid method for the direct determination of formaldehyde in Lentinus edodes. By applying this method to raw and dried samples, it was found that the free formaldehyde content varied with both the strain and the fruiting season. The contents found were higher than those given in other reports and, in the autumnal strains, did not increase after drying. In addition, the formaldehyde formation by enzymatic and acid hydrolysis also depended on the strain and fruiting season.Further studies are required to identify the precursor of formaldehyde in Lentinus edodes.