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Background: Sharing and fairness are important prosocial behaviors that help us navigate the social world. However, little is known about how and whether individuals with Williams Syndrome (WS) engage in these behaviors. The unique phenotype of individuals with WS, consisting of high social motivation and limited social cognition, can also offer insight into the role of social motivation in sharing and fairness when compared to typically developing (TD) individuals. The current study used established experimental paradigms to examine sharing and fairness in individuals with WS and TD individuals. Methods: We compared a sample of patients with WS to TD children (6-year-olds) matched by mental age (MA) on two experimental tasks: the Dictator Game (DG, Experiment 1, N = 17 WS, 20 TD) with adults modeling giving behaviors used to test sharing and the Inequity Game (IG, Experiment 2, N = 14 WS, 17 TD) used to test fairness. Results: Results showed that the WS group behaved similarly to the TD group for baseline giving in the DG and in the IG, rejecting disadvantageous offers but accepting advantageous ones. However, after viewing an adult model giving behavior, the WS group gave more than their baseline, with many individuals giving more than half, while the TD group gave less. Combined these results suggest that social motivation is sufficient for sharing and, in particular, generous sharing, as well as the self-focused form of fairness. Further, individuals with WS appear capable of both learning to be more generous and preventing disadvantageous outcomes, a more complex profile than previously known. Conclusions: In conclusion, the present study provides a snapshot into sharing and fairness-related behaviors in WS, contributing to our understanding of the intriguing social-behavioral phenotype associated with this developmental disorder. [ABSTRACT FROM AUTHOR]

Hair is good bioindicator of exposure, due to its ability to store and retain trace elements for long periods of time. But it can be especially useful when hair dyes are used since they may contain potentially toxic salts in their composition. In this context, analytical methods for the determination of bismuth, cadmium, lead, and silver in scalp human hair by electrothermal atomic absorption spectrometry were successfully validated. A total of 60 samples obtained from women between 18 and 60 years were analyzed: 34 dyed hairs and 26 untreated hairs (control). Average results expressed in dry weight (dyed/control) for each element were 2.34/0.49 μg g−1 (silver), 0.142/0.139 μg g−1 (bismuth), 0.055/0.054 μg g−1 (cadmium), and 2.09/0.99 μg g−1 (lead), respectively. These results agreed with those previously reported for non-exposed populations. A statistically significant higher Ag concentration in dyed hairs was observed, suggesting the bioaccumulation of this element. The associations between metal concentration and variables of interest (age, education, smoking habit, dye brand, use of dietary supplements) were investigated. A strong Pearson correlation was found for the pair Ag/Pb (r = 0.494, p < 0.05). Also, strong associations between lead levels and all the selected variables were observed (p < 0.05), while strong associations between silver levels with age and dye brand and association between cadmium levels and smoking habit were found. Furthermore, several commercial hair dye brands were analyzed to verify compliance with cosmetic regulations. This constitutes the first study of such characteristics performed in Uruguay, with worldwide relevance. [ABSTRACT FROM AUTHOR]

Background: Innovative digital solutions are shaping a new concept of dementia care, opening additional venues for prevention, diagnosis, monitoring and treatment. Hereby, we report the development of a tablet-based teleneuropsychology platform (Tenèpsia®), from concept to certification as Medical Device (MD) Class IIA, as per new MD regulation 745/2017. Methods: The platform was designed for the remote cognitive evaluation and created thanks to the effort of a collaborative working group including experts from three Italian scientific societies and Biogen Italia S.r.l. (hereafter "Biogen"), and developers from Xenia Reply and Inside AI. The development strategy was guided by converting traditional paper-and-pencil tests into digital versions while maintaining comparable neuropsychological features and optimizing patient accessibility and user experience. The experts focused on the choice and adaptation of traditional neuropsychology measures for a 45-min teleneuropsychology assessment. Results: The developers created a web and a mobile interface, respectively, for the professional (neuropsychologist) and non-professional (patient and caregiver) use. Recording of voice, drawing and typing information was enabled. Instant dashboards provide a quick overview of the patient's condition. Simulation activities were performed to obtain MD certification, valid across Europe. Conclusion: Neuropsychology services will benefit from the implementation in clinics of harmonized digital tools with adequate scientific and technological standards. The use of digital cognitive testing for the diagnosis of mild cognitive impairment is expected to enhance patient and clinician outcomes through simplified, digital objective data collection, sparing of time and resources, with a positive impact on healthcare costs and access to treatments, reducing inequalities and delays in diagnosis and cure. [ABSTRACT FROM AUTHOR]

The aim of this work was to study the distribution of relevant inorganic contaminants in environmental samples from Ciudad de la Costa, the second most populated city of Uruguay. For this task aluminum (Al), cadmium (Cd), chromium (Cr), nickel (Ni) and lead (Pb) levels were determined in sand, water, and plant material from six beaches that are located in the area. Analytical methods were successfully validated. Accuracy was ensured by using certified reference materials and spiking procedures. Average metal concentrations in sand and water samples were compared with the maximum limits established by international or national regulations (when available), being far below these limits in all cases. This may indicate that the inorganic contamination in the studied area is rather low, despite the rapid increase in anthropic activities in recent years. To give a deeper characterization of the ecosystem, metal accumulation and mobility within a common plant of the zone, Ammophila arenaria, were studied. Results showed that concentrations in roots were one order of magnitude higher in comparison to aerial parts. In addition, the bioaccumulation coefficient (BAC) showed that the plant accumulates Cd, Cr and Ni, while a low accumulation for Pb was observed. Finally, a Cr(VI) speciation analysis in Ammophila arenaria was performed, showing Cr(VI) percentages below 4.6%, which is relevant from the (eco)toxicological point of view due to the high environmental mobility and bioavailability of this valence state. This work constitutes the first environmental surveillance report of this kind, regarding this populated area of the country. [ABSTRACT FROM AUTHOR]

The epigenetic role of microRNAs is established at both physiological and pathological levels. Dysregulated miRNAs and their targets appear to be a promising approach for innovative anticancer therapies. In our previous study, circulating miR-197-3p tested dysregulated in workers ex-exposed to asbestos (WEA). Herein, an epigenetic investigation on this circulating miRNA was carried out in sera from malignant pleural mesothelioma (MPM) patients. MiR-197-3p was quantified in MPM (n = 75) sera and comparatively analyzed to WEA (n = 75) and healthy subject (n = 75) sera, using ddPCR and RT-qPCR techniques. Clinicopathological characteristics, occupational, non-occupational information and overall survival (OS) were evaluated in correlation studies. MiR-197-3p levels, analyzed by ddPCR, were significantly higher in MPM than in WEA cohort, with a mean copies/µl of 981.7 and 525.01, respectively. Consistently, RT-qPCR showed higher miR-197-3p levels in sera from MPM with a mean copies/µl of 603.7, compared to WEA with 336.1 copies/µl. OS data were significantly associated with histologic subtype and pleurectomy. Circulating miR-197-3p is proposed as a new potential biomarker for an early diagnosis of the MPM onset. Indeed, miR-197-3p epigenetic investigations along with chest X-ray, computed tomography scan and spirometry could provide relevant information useful to reach an early and effective diagnosis for MPM. [ABSTRACT FROM AUTHOR]

An analytical method for sample preparation and subsequent determination of Cd, Pb and Se in beef using aerosol dilution by inductively coupled plasma-mass spectrometry was developed. The use of high matrix introduction technology allowed the introduction of a complex sample into the plasma with higher content of total dissolved solids a residual acidity of 4% and better tolerance to matrix effects. Trueness was verified by the analysis of two reference materials. At the 95% significance level, results were statistically equivalent to the certified or informed values. Analytical precision expressed as relative standard deviation was less than 9% in all cases. After validation, a total of 12 beef samples from Uruguay were analyzed. Obtained levels for Cd and Pb were far below the maximum limits established by international regulations in all samples. Also, results showed that beef from this region is a good source of Se in the range 0.120–0.870 mg kg−1 with an interesting contribution to the daily intake of this essential element. [ABSTRACT FROM AUTHOR]

An alkaline sample treatment using tetramethylammonium hydroxide was optimized and validated for the determination of Cu, Mo, and Zn in beef samples from Uruguay, aiming to contribute to the food quality evaluation of this product. The optimization of the developed method was performed by means of multivariate experiments. Copper and Zn were determined by flame atomic absorption spectrometry and Mo was determined by electrothermal atomic absorption spectrometry. The proposed method consisted of adding 5.0 mL of ultrapure water to 0.25 g of the sample, followed by 1.5 mL of tetramethylammonium hydroxide 25% w w−1 and 60 min at 85 °C in a water bath. After centrifuging, the obtained solution can be used for the analytical determinations of the three elements. Certified reference materials were analyzed and a comparison with microwave-assisted acid digestion was performed to guarantee reliability. At the 95% significance level, the obtained results were statistically equivalent to the certified values and to those obtained by total acid digestion for all the studied elements. Analytical precision expressed as relative standard deviation was less than 8% in all cases. Copper, Mo, and Zn were determined in Uruguayan beef samples using the proposed alkaline method. Besides, novel information concerning Mo levels was presented in this work. [ABSTRACT FROM AUTHOR]

From January 2021, 2 months after CAR-T treatment, the blood cell count revealed several episodes of cyclic neutropenia without consequences that did not need specific treatment. Dear Editor, CD19-targeted chimeric antigen receptor-modified T-cell (CAR-T cell) immunotherapy has shown excellent anti-tumor activity in patients with relapsed/refractory acute lymphoblastic leukemia (ALL) [[1]] and non-Hodgkin lymphoma (NHL) [[2]], which led to the approval of tisagenlecleucel and axicabtagene ciloleucel by the USA Food and Drug Administration (FDA). [Extracted from the article]

Background: Merkel cell carcinoma (MCC) is an uncommon, aggressive malignancy of the skin, mostly affecting head and neck area in elderly white patients. Between head/neck sites, face accounts for 61% and forehead accounts for 17% of all face MCCs. Purpose: We here present a literature review MCC cases arising in the forehead area, published in the English literature in the period 1987–2018, and report a personal observation with a late diagnosis and a treatment out of the current recommendations. The aims of this paper are to provide an up-to-date on MCC arising in the forehead area and to raise awareness about misdiagnosis of this type of lesion mimicking arteriovenous malformations (AVM). Material and method: Literature review was performed on PubMed and Medline database and "Merkel cell carcinoma (MCC)," "forehead" and "MCC forehead location" were the terms the authors searched for. Patients' data have been drawn from descriptions of single cases and of short case series reports. For each case, data were collected about clinical characteristics, treatment modalities and outcomes. The study has been limited to the clinical features of the disease, excluding etiologic/pathogenic aspects. Results: Twenty-five patients with forehead MCC have been identified, coming from 20 sources. Nineteen presented a locoregional disease and 6 had an advanced pathology. TNM classification was reported in only two cases lacking for the other available data. Patients presented at mean age of 66 years with solitary or multiple nodules or dome-shaped/hemispherical mass, rarely ulcerated. Mean size of tumors was 1.13 cm of max diameter. Previous or concurrent malignancies or immune-hematologic disorders (AIDS) were often associated. At first investigation, lesion was often mistaken for other malignant or benign processes and, then, diagnosis was generally late. Some type of preoperative biopsy was performed in 3 patients, while the others had only a postoperative microscopic study of specimen. Initial treatment consisted in 6 cases (24%) in a not further specified about extent and width of margins local excision of the primary lesion, while a wide resection was reported in only 3 cases (12%). Surgical treatment of involved lymph-nodes was performed in 3 cases (12%). Six patients underwent radiotherapy for locoregional or distant recurrences. Mortality and overall survival rate at five years were 28% and 24%, respectively. Spontaneous regression was observed in 3 patients (12%). Case report: Personal observation concerned an 82-year-old woman presenting with a forehead periorbital 5 × 5 cm red-bluish mass. The erythematous lesion was erroneously diagnosed as hemangioma on the base of color, the absence of any signs of malignancy, an angio CT indicating a hypervascular tissue and a FNA cytology (FNAC) lacking of malignant cells. The mass was excised as a benign lesion with about 1 cm margins extent without searching larger edges. Postoperative radiotherapy was offered to the patient after histology report, but she refused. After 4 months from surgery, she had a parotid metastasis and died from the illness in spite of platinum-based chemotherapy. Conclusions: This study confirms the aggressiveness of forehead MCC, comparable with that of other face similar tumors. Personal case suggests that the deceitful benign feature of lesion may mimic an AVM and that FNAC may be misleading and diagnostic failure worsen prognosis. Our experience suggests that in the face smaller than 2–3 cm margins resection may increase the risk of locoregional recurrence. Therefore, postoperative wide-field irradiation should be ever delivered, after forehead MCC surgery, not only when clear margins are unattainable or involved with tumor, but also when negative microscopic edges are documented and residual cancer is thought not persist in the tumor bed. Orbit irradiation seems to be not dangerous for the eye. [ABSTRACT FROM AUTHOR]

A multigenic locus at 3p13-14, spanning FOXP1 to SHQ1, is commonly deleted in prostate cancer and lost broadly in a range of cancers but has unknown significance to oncogenesis or prognosis. Here, we report that FOXP1-SHQ1 deletion cooperates with PTEN loss to accelerate prostate oncogenesis and that loss of component genes correlates with prostate, breast, and head and neck cancer recurrence. We demonstrate that Foxp1-Shq1 deletion accelerates prostate tumorigenesis in mice in combination with Pten loss, consistent with the association of FOXP1-SHQ1 and PTEN loss observed in human cancers. Tumors with combined Foxp1-Shq1 and Pten deletion show increased proliferation and anaplastic dedifferentiation, as well as mTORC1 hyperactivation with reduced Akt phosphorylation. Foxp1-Shq1 deletion restores expression of AR target genes repressed in tumors with Pten loss, circumventing PI3Kmediated repression of the androgen axis. Moreover, FOXP1-SHQ1 deletion has prognostic relevance, with cancer recurrence associated with combined loss of PTEN and FOXP1-SHQ1 genes. [ABSTRACT FROM AUTHOR]

Half of all prostate cancers are caused by the TMPRSS2-ERG gene-fusion, which enables androgens to drive expression of the normally silent E26 transformation-specific (ETS) transcription factor ERG in prostate cells. Recent genomic landscape studies of such cancers have reported recurrent point mutations and focal deletions of another ETS member, the ETS2 repressor factor ERF. Here we show these ERF mutations cause decreased protein stability and mostly occur in tumours without ERG upregulation. ERF loss recapitulates the morphological and phenotypic features of ERG gain in normal mouse prostate cells, including expansion of the androgen receptor transcriptional repertoire, and ERF has tumour suppressor activity in the same genetic background of Pten loss that yields oncogenic activity by ERG. In the more common scenario of ERG upregulation, chromatin immunoprecipitation followed by sequencing indicates that ERG inhibits the ability of ERF to bind DNA at consensus ETS sites both in normal and in cancerous prostate cells. Consistent with a competition model, ERF overexpression blocks ERG-dependent tumour growth, and ERF loss rescues TMPRSS2-ERG-positive prostate cancer cells from ERG dependency. Collectively, these data provide evidence that the oncogenicity of ERG is mediated, in part, by competition with ERF and they raise the larger question of whether other gain-of-function oncogenic transcription factors might also inactivate endogenous tumour suppressors. [ABSTRACT FROM AUTHOR]

Studies of ETS-mediated prostate oncogenesis have been hampered by a lack of suitable experimental systems. Here we describe a new conditional mouse model that shows robust, homogenous ERG expression throughout the prostate. When combined with homozygous Pten loss, the mice developed accelerated, highly penetrant invasive prostate cancer. In mouse prostate tissue, ERG markedly increased androgen receptor (AR) binding. Robust ERG-mediated transcriptional changes, observed only in the setting of Pten loss, included the restoration of AR transcriptional output and upregulation of genes involved in cell death, migration, inflammation and angiogenesis. Similarly, ETS variant 1 (ETV1) positively regulated the AR cistrome and transcriptional output in ETV1-translocated, PTEN-deficient human prostate cancer cells. In two large clinical cohorts, expression of ERG and ETV1 correlated with higher AR transcriptional output in PTEN-deficient prostate cancer specimens. We propose that ETS factors cause prostate-specific transformation by altering the AR cistrome, priming the prostate epithelium to respond to aberrant upstream signals such as PTEN loss. [ABSTRACT FROM AUTHOR]

Two recent phases of mobilization of a large, rainfall-induced debris slide are analysed in terms of relationships between rains and phases of displacement. The first activation at San Rocco (San Benedetto Ullano, Calabria) occurred on 28 January 2009, after extraordinary rains had stricken the region for a couple of months. Detailed geomorphologic field surveys, combined with measurements of superficial displacements at datum points, were performed to properly recognize the evolution of the phenomenon. In addition, a real-time control system of rains and superficial displacements measured at extensometers was implemented, to better analyse the evolution of the phenomenon. In early May 2009, the activity reduced to very slow displacements, persisting in the same condition for the following 8 months. On 1 February 2010, premonitory signs of a new phase of activation were noticed, again following 2 months of extraordinary rainfalls. After few days of further precipitations, the middle sector of the landslide activated since 11 February, disrupting the road network and threatening the major lifelines and some buildings. A hydrological analysis aimed at simulating the dates of activation and the main phases of acceleration of the phenomenon was carried out, by calibrating the empirical model FLaIR against the daily rainfalls and the history of known phases of mobilization since 1970. Calibration allowed to successfully simulate both the cited phases of activity of the San Rocco landslide, by predicting the beginning of the movements as well as the following paroxysmal stages, as testified by the measurements at datum points and extensometers. The set of parameters obtained through calibration reflects the influence of both prolonged antecedent rains, and of high-intensity rainfalls of shorter duration, which slightly preceded the major displacements. Once calibrated the model, a suitable threshold could be defined, by analysing the trend of the mobility function against the history of activations of the considered slope movement, and by excluding false alarms. Accordingly, a reliable tool for predicting the phases of activity of a large slope movement could therefore be obtained. [ABSTRACT FROM AUTHOR]

In this paper we describe and apply the estimating function methodology to value the risk of asset derivative portfolios. We first implement the Li’s model based on the first four moments and then we show the limits of this model in forecasting the maximum loss of contingent claims. In addition, we show that four moments are not enough to describe the behavior of the lower percentiles of derivatives. Finally, we propose a model that considers the first six moments and we compare the performances of these models proposing a backtest analysis on several historical and truncated asset derivative portfolios. [ABSTRACT FROM AUTHOR]

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This article was developed to assist the regular education elementary classroom teacher in utilizing practical bibliotherapy strategies for the inclusive classroom. Using quality children’s literature for bibliotherapy helps students to grow socio-emotionally by identifying with the main character in the story with a disability which leads to personal insight and growth. Teachers will learn how to use a checklist which was developed by the authors to select appropriate bibliotherapy literature and lead classroom discussions. A top ten list of books on bibliotherapy for the inclusive elementary classroom is provided. [ABSTRACT FROM AUTHOR]

The purpose of this article was to illuminate for early childhood teacher practitioners how guided reading, as a research-based approach to reading instruction, could address the challenges of early reading instruction. The early years are the focus for the prevention of reading difficulties and research conducted over the past two decades has produced extensive results demonstrating that children who get off to a poor start in reading rarely catch up (Lentz, ; Neuman & Dickinson, ; Snow, Burns, & Griffin, ; Torgesen, ; Whitehurst & Lonigan, ). One particular research-based strategy, guided reading, is an important “best practice” associated with today’s balanced literacy instruction. The National Reading Panel () argued that balanced approaches are preferable when teaching children to read, based on their review of scientific research-based reading instructional practices used by teachers in classrooms across the country. Additionally, guided reading practices as part of a balanced literacy program conforms to the recommendations on literacy as suggested in position statements by the International Reading Association/The National Association for the Education of Young Children (), and the National Council of Teachers of English (). [ABSTRACT FROM AUTHOR]