Ocular manifestations in Gorlin-Goltz syndrome

Background: Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare genetic disorder that is transmitted in an autosomal dominant manner with complete penetrance and variable expressivity. It is caused in 85% of the cases with a known etiology by pathogenic variants in the PTCH1 gene, and is characterized by a wide range of developmental abnormalities and a predisposition to multiple neoplasms. The manifestations are multiple and systemic and consist of basal cell carcinomas in various regions, odontogenic keratocistic tumors and skeletal anomalies, to name the most frequent. Despite the scarce medical literature on the topic, ocular involvement in this syndrome is frequent and at the level of various ocular structures. Our study focuses on the visual apparatus and its annexes in subjects with this syndrome, in order to better understand how this syndrome affects the ocular system, and to evaluate with greater accuracy and precision the nature of these manifestations in this group of patients. Results: Our study confirms the presence of the commonly cited ocular findings in the general literature regarding the syndrome [hypertelorism (45.5%), congenital cataract (18%), nystagmus (9%), colobomas (9%)] and highlights strabismus (63% of the patients), epiretinal membranes (36%) and myelinated optic nerve fiber layers (36%) as the most frequent ophthalmological findings in this group of patients. Conclusions: The presence of characteristic and frequent ocular signs in the Gorlin- Goltz syndrome could help with the diagnostic process in subjects suspected of having the syndrome who do not yet have a diagnosis. The ophthalmologist has a role as part of a multidisciplinary team in managing these patients. The ophthalmological follow-up that these patients require, can allow, if necessary, a timely therapy that could improve the visual prognosis of such patients

Leachate analyses of volcanic ashes from Stromboli volcano: A proxy for the volcanic gas plume composition?

Many volcanoes show a change in chemical composition of the gas phase prior to periods of eruptive activity. Fine‐grained tephra erupted from active vents and transported through volcanic plumes can adsorb, and therefore rapidly scavenge, volatile elements such as sulfur, halogens, and metal species in the form of soluble salts adhering to ash surfaces. Analysis of such water‐soluble surface materials is a suitable supplement for remote monitoring of volcanic gases at inaccessible volcanoes. In this work, ash samples of the 2004 to 2009 eruptive activity of Stromboli volcano were sampled, leached, and analyzed for major and trace elements. Data analysis and interpretation was focused on determining the relationship between chemical composition of water‐soluble components adhering to volcanic ash and the volcano’s activity state. First results show significant temporal variations in ash leachate compositions, reflecting changes in the eruptive style of the volcano. In particular, we reveal that ash leachates S/F and Mg/Na ratios showed marked increases prior to a large‐scale explosion on 15 March 2007.PublishedD172041.2. TTC - Sorveglianza geochimica delle aree vulcaniche attiveJCR Journalreserve

From social context and resilience to performance through job satisfaction: A multilevel study over time

Giving the crucial role of organizational context in shaping individual attitudes and behaviors at work, in this research we studied the effects of collective work-unit Perceptions of Social Context (PoSC) on individual work resilience and two key individual outcomes: job satisfaction and job performance as rated by the supervisor. We theorized that collective PoSC act as antecedents of individual variables, and that individual job satisfaction mediates the relationship between collective PoSC and job performance, and between work resilience and job performance over time. A sample of 305 white-collar employees, clustered in 67 work-units, participated in the study. Hierarchical linear modeling highlighted that collective PoSC are significant related to individual work resilience. Moreover, results showed that individual job satisfaction fully mediates the relationship between collective PoSC and individual job performance and the relationship between individual work resilience and individual job performance. At a practical level, results suggest that interventions on collective PoSC may increase work resilience, job satisfaction and job performance over time at the individual level

Evidence for Two Superconducting Energy Gaps in MgB2 by Point-Contact Spectroscopy

International audienceExperimental support is found for the multiband model of the superconductivity in the recently discovered system MgB2 with the transition temperature Tc 39 K. By means of Andreev reflection, evidence is obtained for two distinct superconducting energy gaps. The sizes of the two gaps (DS 2.8 meV and DL 7 meV) are, respectively, smaller and larger than the expected weak coupling value. Because of the temperature smearing of the spectra the two gaps are hardly distinguishable at elevated temperatures, but when a magnetic field is applied the presence of two gaps can be demonstrated close to the bulk Tc in the raw data

Conceptions of art : a case study of elementary teachers, a principal, and an art teacher.

This qualitative case study investigated elementary teachers\u27 concepts of art, their anxieties associated with art practices, and a principal\u27s decision making concerning art in the curriculum. Two in-depth interviews were conducted with 12 participants to determine their knowledge base, family and educational backgrounds, conceptions of art, and the relationship with the art teacher. Observations of classroom art activities were carried out over a period of six months and recorded in a field log. Visual images in the school building were analyzed for content. Data were analyzed through open and pattern coding. Through axial coding, clusters of data were organized by commonalities and patterns arranged around the axis category of teachers\u27 conception of art. Through the metaphor of The Medium of Water representing teachers\u27 understanding of art, six themes were developed explaining the results of this study: (1) Skimming the Surface of the Water---describes classroom teachers\u27 inadequate backgrounds. A lack of exposure, education, and familiarity connected to art knowledge was linked to how art was superficially conceptualized and valued. (2) Wading in the Shallows---describes classroom teachers\u27 shallow conceptions of art. (3) A Choice Not to Dive---describes manifestations of classroom teachers\u27 conceptions of art and implementation of art. Images displayed within classrooms and corridors were found to be predominately commercially adult-generated and/or student-generated from the art curriculum. (4) Fear of the Water---describes anxieties associated with the teaching and making of art. Anxious participants did not consider themselves artistic and used less art in their classrooms. (5) Unable to Take the Plunge---describes a knowledgeable principal\u27s indecision. While being knowledgeable and sympathetic to art, she was unable to make a case for an art-inclusive program, and (6) Drowning in Responsibilities---describes an overwhelmed art teacher. Her isolation contributed to an inability to collaborate with other teachers and responsibility to integrate fell mostly to her. Her teaching objectives and values did not match with classroom teachers\u27. All participants\u27 concerns were coping with curricular pressures, high-stakes testing, and lack of time in the schedule. Because of these issues, art was not a priority in this school\u27s curriculum

Translating self-efficacy in job performance over time: The role of job crafting

This investigation intends to uncover the mechanisms linking self-efficacy to job performance by analyzing the mediating role of job crafting. A two-wave study on 465 white-collar workers was conducted, matching participants’ self-report data (i.e., self-efficacy and job crafting) with supervisory performance ratings. The structural equation model showed a positive reciprocal relationship between self-efficacy and crafting behaviors. In turn, job crafting predicted performance positively over time. More importantly, results confirmed the mediating role of crafting actions, which may represent the behavioral process underlying the positive effect of self-efficacy on individual outcomes. Practical implications for organizations, such as encouraging bottom-up job design or designing job-crafting interventions, and future research directions are also offered