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Langerhana islets are constituted by four different cellular populations with a central core of  cells surrounded by ,  and PP cells. Islets may be dissociated to single component cells that re-establish contacts to form organized and functional aggregates capable of responding appropriately to islet secretory stimuli (1-5). [...]

Despite a high rate of concurrent mathematical difficulties among children with dyslexia, we still have limited information regarding the prevalence and severity of mathematical deficits in this population. To address this gap, we developed a comprehensive battery of cognitive tests, known as the UCSF Mathematical Cognition Battery (MCB), with the aim of identifying deficits in four distinct mathematical domains: number processing, arithmetical procedures, arithmetic facts retrieval, and geometrical abilities. The mathematical abilities of a cohort of 75 children referred to the UCSF Dyslexia Center with a diagnosis of dyslexia, along with 18 typically developing controls aged 7 to 16, were initially evaluated using a behavioral neurology approach. A team of professional clinicians classified the 75 children with dyslexia into five groups, based on parents and teachers reported symptoms and clinical history. These groups included children with no mathematical deficits and children with mathematical deficits in number processing, arithmetical procedures, arithmetic facts retrieval, or geometrical abilities. Subsequently, the children underwent evaluation using the MCB to determine concordance with the clinicians impressions. Additionally, neuropsychological and cognitive standardized tests were administered. Our study reveals that within a cohort of children with dyslexia, 66% exhibit mathematical deficits, and among those with mathematical deficits, there is heterogeneity in the nature of these deficits. If these findings are confirmed in larger samples, they can potentially pave the way for new diagnostic approaches, consistent subtype classification, and, ultimately personalized interventions.

BACKGROUND: Dilated cardiomyopathy (DCM) is a major complication of, and leading cause of mortality in Duchenne muscular dystrophy (DMD). Its severity, age at onset, and rate of progression display wide variability, whose molecular bases have been scarcely elucidated. Potential DCM-modifying factors include glucocorticoid (GC) and cardiological treatments, DMD mutation type and location, and variants in other genes. METHODS AND RESULTS: We retrospectively collected 3138 echocardiographic measurements of left ventricular ejection fraction (EF), shortening fraction (SF), and end-diastolic volume (EDV) from 819 DMD participants, 541 from an Italian multicentric cohort and 278 from the Cooperative International Neuromuscular Group Duchenne Natural History Study (CINRG-DNHS). Using generalized estimating equation (GEE) models, we estimated the yearly rate of decrease of EF (-0.80%) and SF (-0.41%), while EDV increase was not significantly associated with age. Utilizing a multivariate generalized estimating equation (GEE) model we observed that mutations preserving the expression of the C-terminal Dp71 isoform of dystrophin were correlated with decreased EDV (-11.01 mL/m2, p = 0.03) while for dp116 were correlated with decreased EF (-4.14%, p =

Despite a high rate of concurrent mathematical difficulties among children with dyslexia, we still have limited information regarding the prevalence and severity of mathematical deficits in this population. To address this gap, we developed a comprehensive battery of cognitive tests, known as the UCSF Mathematical Cognition Battery (MCB), with the aim of identifying deficits in four distinct mathematical domains: number processing, arithmetical procedures, arithmetic facts retrieval, and geometrical abilities. The mathematical abilities of a cohort of 75 children referred to the UCSF Dyslexia Center with a diagnosis of dyslexia, along with 18 typically developing controls aged 7 to 16, were initially evaluated using a behavioral neurology approach. A team of professional clinicians classified the 75 children with dyslexia into five groups, based on parents' and teachers' reported symptoms and clinical history. These groups included children with no mathematical deficits and children with mathematical deficits in number processing, arithmetical procedures, arithmetic facts retrieval, or geometrical abilities. Subsequently, the children underwent evaluation using the MCB to determine concordance with the clinicians' impressions. Additionally, neuropsychological and cognitive standardized tests were administered. Our study reveals that within a cohort of children with dyslexia, 66% exhibit mathematical deficits, and among those with mathematical deficits, there is heterogeneity in the nature of these deficits. If these findings are confirmed in larger samples, they can potentially pave the way for new diagnostic approaches, consistent subtype classification, and, ultimately personalized interventions.

The purpose of this article is to better understand how proof is introduced into the study of quadrilaterals and triangles in high school. To do this, we designed a grid to analyse mathematics textbooks in Cameroon francophone subsystems (7th Grade and 8th Grade). The Anthropological Theory of Didactics and the paradigms in geometry served as a theoretical framework for our analyses. The results of our analysis indicate that problems in the lessons section correspond to guided problems. These kinds of problems do not develop students' spirit of research and initiative. The authors of the textbook choose to teach the functioning of deductive reasoning in the 8th Grade. They choose to introduce proof in the commented exercise section rather than lessons section. The learning problems proposed in the textbooks contain drawings wish have informative function and representative function. The preponderance of drawings with a representative function that have the same shape and name observed in textbooks can contribute to the construction of constant visual models in students' minds. This could lead to the superficial use of drawings in proof tasks.

Objective/Context: This historiographical introduction, as well as the articles published in this special issue of Historia Crítica entitled “The Chilean Way to Socialism” from a transnational perspective, aim to highlight how this approach helps us to overcome the limitations inherited from the existing historiography on the Unidad Popular (up), which tends to adopt a national framework of analysis. Methodology: It consists of critically analyzing and summarizing the contributions of the research carried out on the up from 2003 to date. Originality and Conclusions: We found that the transnational dimension of the “1970-1973 cycle” has been absent or marginal in much of the historiography about the period. The national approach to the political process of the up is generally accompanied by the conviction about the alleged exceptionality of the “thousand days” of Allende. Based on the historiographical discussion of the present essay and the contributions of the articles in the dossier, we show that the transnational approach to the up allows its complete understanding by inserting it in a broader context than the national one. The regional approach makes it possible to demonstrate that the up is one of the numerous projects of “democratic revolution” that marked the “Inter-American Cold War,” while the global approach shows that the impact of the up was not only due to its exceptional nature but also to the resonance of the “Chilean road to socialism” project, with the long-standing debates on revolutionary paths and models of society that agitated the world political lefts throughout the twentieth and twenty-first centuries. The focus on the “peripheral spaces” of the Cold War allows us to think of the Allende government as an additional case of the successive efforts to find an alternative path to Soviet socialism and u.s. capitalism.

Sleep medicine is a relatively young field with exponential growth in development and research in the last decades. Parallel to the advances in the United States, Latin America also had its beginnings in sleep medicine housed in neuroscience laboratories. Since the very first Latin American meeting in 1985, and the first sleep society in 1993, sleep research has undergone significant development in subsequent years. From contributions in animal research that allowed understanding of the activity of the brain during sleep to the studies that improved our knowledge of sleep disorders in humans, Latin America has become a scientific hub for expansion of sleep research. In this article, we present a historical account of the development of sleep medicine in Latin America, the current state of education and the achievements in research throughout history, and the latest advances in the trending areas of sleep science and medicine. These findings were presented during World Sleep Society meeting in Vancouver in 2019 and complement the work on sleep societies and training published by Vizcarra-Escobar et al in their article Sleep societies and sleep training programs in Latin America (J Clin Sleep Med. 2020;16(6):983-988).

The objective of the study was to determine if a feeding system with a variable supply of grass promoted rapid changes in the fatty acid profile and technological and health indices of milk obtained from North American (NAHF) and New Zealand (NZHF) Holstein-Friesian cows. Two feeding strategies were conducted: fixed grass (GFix) and maximized grass intake when available (GMax). The results showed that as the grass intake increased in the GMax treatments, the relative amount of palmitic acid in milk decreased, whereas oleic, linoleic, linolenic, and conjugated linoleic acids increased, causing a reduction in the atherogenic, thrombogenic, and spreadability calculated indices. The changes occurred in rapid response to the changing diet, with reductions ranging from approximately 5 to 15% in the healthy and technological indices within a period of 15 d of grass intake increase. Differences were found between the 2 genotypes, with NZHF responding faster to changes in grass intake.

Abstract Cities in the global south face dire climate impacts. It is in socioeconomically marginalized urban communities of the global south that the effects of climate change are felt most deeply. Santiago de Chile, a major mid-latitude Andean city of 7.7 million inhabitants, is already undergoing the so-called “climate penalty” as rising temperatures worsen the effects of endemic ground-level ozone pollution. As many cities in the global south, Santiago is highly segregated along socioeconomic lines, which offers an opportunity for studying the effects of concurrent heatwaves and ozone episodes on distinct zones of affluence and deprivation. Here, we combine existing datasets of social indicators and climate-sensitive health risks with weather and air quality observations to study the response to compound heat-ozone extremes of different socioeconomic strata. Attributable to spatial variations in the ground-level ozone burden (heavier for wealthy communities), we found that the mortality response to extreme heat (and the associated further ozone pollution) is stronger in affluent dwellers, regardless of comorbidities and lack of access to health care affecting disadvantaged population. These unexpected findings underline the need of a site-specific hazard assessment and a community-based risk management.

The impact of ancient urban occupations on sustainability has recently become a topic of interest for archaeologists as well as many other scholars. Much of this archaeological research has focused on documenting the longevity of ancient cities and elucidating the social and economic strategies employed at the urban and regional scales to promote urban sustainability. In this article, we add to this discussion by addressing the issue of sustainability by considering the impact of environmental legacies left by ancient cities after their abandonment. Using a series of cases from pre-Hispanic Mesoamerica [OMYK (Coba), Kiuic, Lagunas de Yalahau, and Tlaxcallan], we show through ethnoecological and historical ecological research that in some cases pre-Hispanic people, living in urban zones, affected “intermediate” disturbances that increased biodiversity, biomass, and sustainability by creating second natures that have endured for centuries.

Abstract Background Pathogenic variants in PEX-genes can affect peroxisome assembly and function and cause Zellweger spectrum disorders (ZSDs), characterized by variable phenotypes in terms of disease severity, age of onset and clinical presentations. So far, defects in at least 15 PEX-genes have been implicated in Mendelian diseases, but in some of the ultra-rare ZSD subtypes genotype–phenotype correlations and disease mechanisms remain elusive. Methods We report five families carrying biallelic variants in PEX13. The identified variants were initially evaluated by using a combination of computational approaches. Immunofluorescence and complementation studies on patient-derived fibroblasts were performed in two patients to investigate the cellular impact of the identified mutations. Results Three out of five families carried a recurrent p.Arg294Trp non-synonymous variant. Individuals affected with PEX13-related ZSD presented heterogeneous clinical features, including hypotonia, developmental regression, hearing/vision impairment, progressive spasticity and brain leukodystrophy. Computational predictions highlighted the involvement of the Arg294 residue in PEX13 homodimerization, and the analysis of blind docking predicted that the p.Arg294Trp variant alters the formation of dimers, impairing the stability of the PEX13/PEX14 translocation module. Studies on muscle tissues and patient-derived fibroblasts revealed biochemical alterations of mitochondrial function and identified mislocalized mitochondria and a reduced number of peroxisomes with abnormal PEX13 concentration. Conclusions This study expands the phenotypic and mutational spectrum of PEX13-related ZSDs and also highlight a variety of disease mechanisms contributing to PEX13-related clinical phenotypes, including the emerging contribution of secondary mitochondrial dysfunction to the pathophysiology of ZSDs.

OBJECTIVE: Respiratory insufficiency is a major complication of Duchenne muscular dystrophy (DMD). Its progression shows considerable interindividual variability, which has been less thoroughly characterized and understood than in skeletal muscle. We collected pulmonary function testing (PFT) data from a large retrospective cohort followed at Centers collaborating in the Italian DMD Network. Furthermore, we analyzed PFT associations with different DMD mutation types, and with genetic variants in SPP1, LTBP4, CD40, and ACTN3, known to modify skeletal muscle weakness in DMD. Genetic association findings were independently validated in the Cooperative International Neuromuscular Research Group Duchenne Natural History Study (CINRG-DNHS). METHODS AND RESULTS: Generalized estimating equation analysis of 1852 PFTs from 327 Italian DMD patients, over an average follow-up time of 4.5 years, estimated that forced vital capacity (FVC) declined yearly by -4.2%, forced expiratory volume in 1 sec by -5.0%, and peak expiratory flow (PEF) by -2.9%. Glucocorticoid (GC) treatment was associated with higher values of all PFT measures (approximately + 15% across disease stages). Mutations situated 3 of DMD intron 44, thus predicted to alter the expression of short dystrophin isoforms, were associated with lower (approximately -6%) PFT values, a finding independently validated in the CINRG-DNHS. Deletions amenable to skipping of exon 51 and 53 were independently associated with worse PFT outcomes. A meta-analysis of the two cohorts identified detrimental effects of SPP1 rs28357094 and CD40 rs1883832 minor alleles on both FVC and PEF. INTERPRETATION: These findings support GC efficacy in delaying respiratory insufficiency, and will be useful for the design and interpretation of clinical trials focused on respiratory endpoints in DMD.

We assess the prospects for central banks using inflation expectations as a policy tool for stabilization purposes. We review recent work on how expectations of agents are formed and how they affect their economic decisions. Empirical evidence suggests that inflation expectations of households and firms affect their actions but the underlying mechanisms remain unclear, especially for firms. Two additional limitations prevent policy-makers from being able to actively manage inflation expectations. First, available surveys of firms' expectations are systematically deficient, which can only be addressed through the creation of large, nationally representative surveys of firms. Second, neither households' nor firms' expectations respond much to monetary policy announcements in low-inflation environments. We provide suggestions for how monetary policy-makers could pierce this veil of inattention through new communication strategies as well as the potential pitfalls to trying to do so.

We assess the prospects for central banks using inflation expectations as a policy tool for stabilization purposes. We review recent work on how expectations of agents are formed and how they affect their economic decisions. Empirical evidence suggests that inflation expectations of households and firms affect their actions but the underlying mechanisms remain unclear, especially for firms. Two additional limitations prevent policy-makers from being able to actively manage inflation expectations. First, available surveys of firms' expectations are systematically deficient, which can only be addressed through the creation of large, nationally representative surveys of firms. Second, neither households' nor firms' expectations respond much to monetary policy announcements in low-inflation environments. We provide suggestions for how monetary policy-makers could pierce this veil of inattention through new communication strategies as well as the potential pitfalls to trying to do so.