A Fuzzy Set Approach to Measuring Violence Against Women and Its Severity

We develop a scale of severity of violence against women based on fuzzy set theory. The scale can be used to derive fuzzy indexes of violence which account for the prevalence, frequency and severity of violence. Using the results of the survey conducted by the European Agency for Human Rights (FRA) we find strong congruence of ranking between the proposed scale and three widely used alternatives – the Conflict Tactic Scale, The Severity of Violence Against Women Scale and the Index of Spouse Abuse. Unlike existing alternatives, however, the scale that we propose is based on objective information rather than subjective assessment; it is parsimonious in terms of the amount of information that it requires; and it is less vulnerable to risks of cultural bias. As an example of the uses to which fuzzy measurement of violence can be put, we compute fuzzy indexes of intimate partner violence for European countries and find a clear, inverse correlation across countries with the degree of gender equality

Maladaptation in an unequal world: an evolutionary model with heterogeneous agents

Maladaptation is steadily increasing its presence in agenda and debates about climate change and its impacts. The term denotes actions undertaken, at the individual or collective level, to defend against the adverse effects of climate change or environmental degradation, but that ultimately exacerbate the underlying risk factors. In this paper, we investigate the effects of maladaptation in terms of well-being and inequality in a two-population (North-South) evolutionary model. While agents in the South often face higher vulnerability to environmental degradation and limited defense mechanisms compared to their Northern counterparts, the latter stand to endure greater economic losses, in absolute terms. Our model demonstrates that the diffusion of maladaptive choices could result in a Pareto-dominated steady state, influencing inequality levels positively or negatively based on the scale of maladaptation impacts relative to the existing environmental degradation. These findings stress the imperative of integrating environmental risk studies with maladaptive effects and dynamics. Additionally, they advocate for international discourse not only on climate change mitigation but also on adaptive measures among countries

Neurological assessment of newborns with spinal muscular atrophy identified through neonatal screening

The possibility to identify patients with spinal muscular atrophy through neonatal screenings has highlighted the need for clinical assessments that may systematically evaluate the possible presence of early neurological signs. The aim of this study was to use the Hammersmith Neonatal Neurological Examination (HNNE) and a module specifically designed for floppy infants to assess the possible variability of neurological findings in infants identified through neonatal screening. The infants included in this study were identified as part of a pilot study exploring neonatal screening in two Italian regions. A neurological examination was performed using the HNNE and an additional module developed for the assessment of floppy infants. Seventeen infants were identified through the screening. One patient had 1 SMN2 copy, 9 had 2 copies, 3 had 3, and 4 had more than 3 copies. Nine of the 17 infants (53%) had completely normal results on both scales, 3 had minimal signs, and the other 5 had more obvious clinical signs. The number of SMN2 copies was related to the presence of abnormal neurological signs (p = 0.036) but two SMN2 copies were associated with variable clinical signs as they were found in some infants with respectively normal examination or obvious severe early signs. Conclusions: Our results suggest that the combination of both scales increases the possibility to detect neonatal neurological signs and to define different early patterns of involvement also identifying paucisymptomatic patients.What is Known:• The use of new therapeutic options in presymptomatic SMA patients leads to a dramatic reduction of the onset and severity of the diesease.• The already existing tools commonly used in Type I SMA (HINE and CHOP-intend) may not be suitable to identify minor neurological signs in the neonatal period.What is New:• Combining the HNNE and the floppy infant module, we were able to identify early neurological signs in SMA infants identified through newborn screening and may help to predict the individual therapeutic outcome of these patients.• Iinfants with 2 SMN2 copies identified through the screening had a more variable neonatal examination compared to those with three or more copies, in agreement with similar findings in older infants

Deoxyguanosine kinase deficiency: natural history and liver transplant outcome

Autosomal recessive pathogenetic variants in the DGUOK gene cause deficiency of deoxyguanosine kinase activity and mitochondrial deoxynucleotides pool imbalance, consequently, leading to quantitative and/or qualitative impairment of mitochondrial DNA synthesis. Typically, patients present early-onset liver failure with or without neurological involvement and a clinical course rapidly progressing to death. This is an international multicentre study aiming to provide a retrospective natural history of deoxyguanosine kinase deficient patients. A systematic literature review from January 2001 to June 2023 was conducted. Physicians of research centres or clinicians all around the world caring for previously reported patients were contacted to provide followup information or additional clinical, biochemical, histological/histochemical, and molecular genetics data for unreported cases with a confirmed molecular diagnosis of deoxyguanosine kinase deficiency. A cohort of 202 genetically confirmed patients, 36 unreported, and 166 from a systematic literature review, were analyzed. Patients had a neonatal onset (<= 1 month) in 55.7% of cases, infantile (>1 month and <= 1 year) in 32.3%, pediatric (>1 year and <= 18 years) in 2.5% and adult (>18 years) in 9.5%. Kaplan-Meier analysis showed statistically different survival rates (P < 0.0001) among the four age groups with the highest mortality for neonatal onset. Based on the clinical phenotype, we defined four different clinical subtypes: hepatocerebral (58.8%), isolated hepatopathy (21.9%), hepatomyoencephalopathy (9.6%), and isolated myopathy (9.6%). Muscle involvement was predominant in adult-onset cases whereas liver dysfunction causes morbidity and mortality in early-onset patients with a median survival of less than 1 year. No genotype-phenotype correlation was identified. Liver transplant significantly modified the survival rate in 26 treated patients when compared with untreated. Only six patients had additional mild neurological signs after liver transplant. In conclusion, deoxyguanosine kinase deficiency is a disease spectrum with a prevalent liver and brain tissue specificity in neonatal and infantile-onset patients and muscle tissue specificity in adult-onset cases. Our study provides clinical, molecular genetics and biochemical data for early diagnosis, clinical trial planning and immediate intervention with liver transplant and/or nucleoside supplementation

Mining and local economies: Dilemma between environmental protection and job opportunities

Mining areas often experience a climate of social tension due to the potential trade-off between expected employment impact and concerns for environmental damage. We address this topic from a theoretical perspective that, unlike most empirical research, includes medium-term dynamics. We developed a two-sector dynamic model that provides a new way to identify differences among mining regions in terms of conflict risk, local development, and welfare. There are critical points in the natural-resource base of local nonmining activities and in the pollution rate of mining operations, which determine the type of dynamics and its welfare outcomes due to the opening up of the economy to mining investment. Pollution control is a sine qua non for welfare gains despite new job opportunities in the mining sector

Modeling maladaptation in the inequality–environment nexus

Adaptation against environmental degradation has the potential to generate further environmental pressures. Does this aspect of adaptation affect the inequality–environment link? To answer this question, we develop a one-sector and one-input model which integrates threats to social and environmental sustainability posed by feedback effects of agents’ adaptation strategies. We distinguish between income inequality and welfare inequality with the latter depending on environmental quality, leisure time, income level and allocation of income to consumption or adaptation. Despite its parsimony, the model describes the conditions for the existence of different inequality–environment dynamic regimes. The model confirms the standard view that environmental degradation exacerbates welfare inequality, but it also produces non-trivial and surprising insights. It illustrates that income inequality affects the type of dynamic regime followed by the economy. High-income economies and economies with high-income inequality are most at risk of following a pattern of maladaptive growth with increasing welfare inequality and environmental pressure