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Background: Patients with sporadic neuroendocrine neoplasms may exhibit a higher risk of a second primary tumor than the general population. Aim: This study aimed to analyze the occurrence of second primary malignancies. Methods: A retrospective cohort of 2757 patients with sporadic lung and gastro-entero-pancreatic neuroendocrine neoplasms, managed at eight Italian tertiary referral Centers, was included. Results: Between 2000 and 2019, a second primary malignancy was observed in 271 (9.8%) neuroendocrine neoplasms patients with 32 developing a third tumor. There were 135 (49.8%) females and the median age was 64 years. The most frequent locations of the second tumors were breast (18.8%), prostate (12.5%), colon (9.6%), blood tumors (8.5%), and lung (7.7%). The second primary tumor was synchronous in 19.2% of cases, metachronous in 43.2%, and previous in 37.6%. As concerned the neuroendocrine neoplasms, the 5- and 10-year survival rates were 87.8% and 74.4%, respectively. PFS for patients with a second primary malignancy was shorter than for patients without a second primary malignancy. Death was mainly related to neuroendocrine neoplasms. Conclusion: In NEN patients the prevalence of second primary malignancies was not negligible, suggesting a possible neoplastic susceptibility. Overall survival was not affected by the occurrence of a second primary malignancy.

Knowledge about the quality of data and metadata is important to support informed decisions on the (re)use of individual datasets and is an essential part of the ecosystem that supports open science. Quality assessments reflect the reliability and usability of data. They need to be consistently curated, fully traceable, and adequately documented, as these are crucial for sound decision-and policy-making efforts that rely on data. Quality assessments also need to be consistently represented and readily integrated across systems and tools to allow for improved sharing of information on quality at the dataset level for individual quality attribute or dimension. Although the need for assessing the quality of data and associated information is well recognized, methodologies for an evaluation framework and presentation of resultant quality information to end users may not have been comprehensively addressed within and across disciplines. Global interdisciplinary domain experts have come together to systematically explore needs, challenges and impacts of consistently curating and representing quality information through the entire lifecycle of a dataset. This paper describes the findings of that effort, argues the importance of sharing dataset quality information, calls for community action to develop practical guidelines, and outlines community recommendations for developing such guidelines. Practical guidelines will allow for global access to and harmonization of quality information at the level of individual Earth science datasets, which in turn will support open science.

ClC-7 is a chloride-proton antiporter of the CLC protein family. In complex with its accessory protein Ostm-1, ClC-7 localizes to lysosomes and to the osteoclasts' ruffled border, where it plays a critical role in acidifying the resorption lacuna during bone resorption. Gene inactivation in mice causes severe osteopetrosis, neurodegeneration, and lysosomal storage disease. Mutations in the human CLCN7 gene are associated with diverse forms of osteopetrosis. The functional evaluation of ClC-7 variants might be informative with respect to their pathogenicity, but the cellular localization of the protein hampers this analysis. Here we investigated the functional effects of 13 CLCN7 mutations identified in 13 new patients with severe or mild osteopetrosis and a known ADO2 mutation. We mapped the mutated amino acid residues in the homology model of ClC-7 protein, assessed the lysosomal colocalization of ClC-7 mutants and Ostm1 through confocal microscopy, and performed patch-clamp recordings on plasma-membrane-targeted mutant ClC-7. Finally, we analyzed these results together with the patients' clinical features and suggested a correlation between the lack of ClC-7/Ostm1 in lysosomes and severe neurodegeneration. © 2020 American Society for Bone and Mineral Research (ASBMR).

ClC-7 is a chloride-proton antiporter of the CLC protein family. In complex with its accessory protein Ostm-1, ClC-7 localizes to lysosomes and to the osteoclasts' ruffled border, where it plays a critical role in acidifying the resorption lacuna during bone resorption. Gene inactivation in mice causes severe osteopetrosis, neurodegeneration, and lysosomal storage disease. Mutations in the human CLCN7 gene are associated with diverse forms of osteopetrosis. The functional evaluation of ClC-7 variants might be informative with respect to their pathogenicity, but the cellular localization of the protein hampers this analysis. Here we investigated the functional effects of 13 CLCN7 mutations identified in 13 new patients with severe or mild osteopetrosis and a known ADO2 mutation. We mapped the mutated amino acid residues in the homology model of ClC-7 protein, assessed the lysosomal colocalization of ClC-7 mutants and Ostm1 through confocal microscopy, and performed patch-clamp recordings on plasma-membrane-targeted mutant ClC-7. Finally, we analyzed these results together with the patients' clinical features and suggested a correlation between the lack of ClC-7/Ostm1 in lysosomes and severe neurodegeneration. © 2020 American Society for Bone and Mineral Research (ASBMR).

This article reports the development and psychometric requisites of Visions About Future (VAF), a measure to assess optimism, pessimism, and hope in early adolescence. Two different studies involving Italian middle school students were conducted. With the first study, the items were developed and the factor structure verified by using item analysis, exploratory factor analysis (EFA), and confirmatory factor analysis (CFA); further, convergent and concurrent validity by testing hypothesised relationships with existing measures, were assessed. In the second study, we provided measurement invariance across gender among middle school students. Results provide strong psychometric support for VAF as a valid measure to explore propensity to optimism, pessimism and hope in early adolescence and for career education and career counselling activities.

During preadolescence children start to give attention to the future, to formulate thoughts, and to wonder about what they want. They also acquire knowledge and experiment with strategies to assist them in designing their future career. Among the constructs that can now play a relevant role in life designing, the four resources of career adaptability have a prominent role. This article reports the psychometric properties of the Italian version of the Career Adapt-Abilities Scale-International Form 2.0 for a group of middle school students. Two different studies involving Italian preadolescents were conducted. The first study tests the instrument's multidimensional structure, internal consistency and discriminant validity. The second one verifies the factorial structure's across-gender invariance and the test-retest reliability. Results provide strong psychometric support for the CAAS-Italian Form with preadolescents as a valid measure for analyzing their career adaptability and for using it in career counseling and education.

The authors examined the relationship between career adaptability, future orientation, and vocational interests. Specifically, they tested the role of career adaptability and future orientation in promoting a broader range of vocational interests, based on the principle that experiencing more interests can be useful in giving one's best in multiple occupations and in planning a broad range of career goals. A total of 762 Italian adolescents (383 males, 379 females) participated in the study. Results highlight the mediating role of future orientation in the relationship between 4 career adaptability resources and breadth of vocational interests. These results underscore that specific interventions in career adaptability and future orientation may foster a broader range of vocational interests, providing more opportunity for adolescents to respond to the demands of the current job market.

Background: Parents of children with intellectual disability (ID) have been found to be more likely to experience lower levels of life satisfaction than parents of typically developing children as a result of the increased challenges they experience. Based on a life design approach, which emphasises the role of career adaptability and resilience in dealing with life challenges, this study aimed at analysing the relationship between career adaptability and life satisfaction through the mediational role of resilience in parents of children with mild ID. Method: One hundred and fifty-two (62 fathers and 90 mothers) parents of children with mild ID were involved and were administered measures of life satisfaction, career adaptability, and resilience. Results: The structural equation model showed that career adaptability is indirectly, through resilience, related to life satisfaction. Conclusion: This result has important implications for practice, and it underscores the need to support parents’ life satisfaction, promoting their resilience, and especially their career adaptability.

Unlike those of past generations, parents today must navigate their children’s education within increasingly complex social environments and during a time of widespread socioeconomic challenges. This complexity social and economic situation lays it on the line some people in respect to others, and among these there are the parents with disability. Past studies of parents of children with disability often contributed to disseminate, as regarded their children as well, negative views of their conditions and perspectives. However, across the past several decades, a new focus has emerged on the positive impact of raising a child with disability. In this chapter, we focus on the roles played by parents of children with and without disability, believing that parents can and should become co-constructing agents of inclusion. We suggest that positive outcomes are influenced by parent attributes of hope, optimism, and courage, and present a number of resources and strategies that can be used to assess and develop these parent strengths, and promote more inclusive attitudes and environments for all.

A career intervention based on life design approach was devised for a group of young adults at risk for the process of career construction. It was aimed at fostering a series of resources useful to cope with career transitions, to encourage reflection on the future, to identify one’s own strengths, and to plan future projects. Results of the study provided evidence for effectiveness of the career intervention across at several methods of change assessment (statistical significance, clinical significance, and social validity) and highlighted the potential of the career intervention.

An increasing number of studies has been conducted on preadolescents development adopting a “positive view” of them rather than focusing on problems they may encounter while growing up. Three different perspectives have been evidenced in the literature and summarized in this paper, namely Positive Youth Development (PYD) which has its roots in the Developmental System Theory, Positive Psychology with attention to values and strengths and, thirdly, Life Design a recently developed approach in career counseling studies. With the analysis of studies carried out with preadolescents according to each of the considered perspectives, the relevance of addressing positive characteristics of preadolescents and fostering their development is underlined. Relevant interventions adopting a positive approach are described with an emphasis on positive changes prompted by these activities during preadolescence. Finally, conclusions about effectiveness of positive, strength based interventions and on their potential in prevention are proposed with suggestions for future programs.

The study is aimed at providing the development and initial validation of the Design My Future (DMF), which may be administered in career counseling and research activities to assess adolescents' future orientation and resilience. Two studies with two independent samples of Italian adolescents were conducted to examine psychometric requisites of DMF. Specifically, in the first study, after developing items and examined the content validity, the factorial structure, reliability and discriminant validity of the DMF were tested. In the second study, the measurement invariance across gender, conducing a sequence of nested CFA models, was evaluated. Results showed good psychometric support for the instrument with Italian adolescents.