The Ethics of Delusional Belief

In this paper we address the ethics of adopting delusional beliefs and we apply consequentialist and deontological considerations to the epistemic evaluation of delusions. Delusions are characterised by their epistemic shortcomings and they are often defined as false and irrational beliefs. Despite this, when agents are overwhelmed by negative emotions due to the effects of trauma or previous adversities, or when they are subject to anxiety and stress as a result of hypersalient experience, the adoption of a delusional belief can prevent a serious epistemic harm from occurring. For instance, delusions can allow agents to remain in touch with their environment overcoming the disruptive effect of negative emotions and anxiety. Moreover, agents are not blameworthy for adopting their delusions if their ability to believe otherwise is compromised. There is evidence suggesting that no evidence-related action that would counterfactually lead them to believe otherwise is typically available to them. The lack of ability to believe otherwise, together with some other conditions, implies that the agents are not blameworthy for their delusions. The examination of the epistemic status of delusions prompts us to acknowledge the complexity and contextual nature of epistemic evaluation, establish connections between consequentialist and deontological frameworks in epistemology, and introduce the notion of epistemic innocence into the vocabulary of epistemic evaluatio

PPA2-associated sudden cardiac death: extending the clinical and allelic spectrum in 20 new families

Purpose Biallelic hypomorphic variants in PPA2, encoding the mitochondrial inorganic pyrophosphatase 2 protein, have been recently identified in individuals presenting with sudden cardiac death, occasionally triggered by alcohol intake or a viral infection. Here we report 20 new families harboring PPA2 variants. Methods Synthesis of clinical and molecular data concerning 34 individuals harboring five previously reported PPA2 variants and 12 novel variants, 11 of which were functionally characterized. Results Among the 34 individuals, only 6 remain alive. Twenty-three died before the age of 2 years while five died between 14 and 16 years. Within these 28 cases, 15 died of sudden cardiac arrest and 13 of acute heart failure. One case was diagnosed prenatally with cardiomyopathy. Four teenagers drank alcohol before sudden cardiac arrest. Progressive neurological signs were observed in 2/6 surviving individuals. For 11 variants, recombinant PPA2 enzyme activities were significantly decreased and sensitive to temperature, compared to wild-type PPA2 enzyme activity. Conclusion We expand the clinical and mutational spectrum associated with PPA2 dysfunction. Heart failure and sudden cardiac arrest occur at various ages with inter- and intrafamilial phenotypic variability, and presentation can include progressive neurological disease. Alcohol intake can trigger cardiac arrest and should be strictly avoided

How do school resources and academic performance differ across Australia’s rural, regional and metropolitan communities?

This study uses data from the Programme for International Student Assessment (PISA) to gain a better understanding of how academic performance and resources vary across rural–urban school communities in Australia. While it is well known that schools in rural areas have difficulty recruiting and retaining teachers, the degree to which schools in larger sized communities across Australia also face this problem is less understood. Moreover, very little is known about the degree to which shortages of instructional materials and equipment are associated with rural–urban location. The analysis includes 353 schools across eight community types that range in size of <1,000 people in small country towns to more than a million people in large capital cities. School principals reported the degree to which instruction in their school is hindered by a shortage of resources, which include qualified teaching staff and instructional materials and equipment. The findings highlight the extent to which school resources vary across geographic location, as reported by school principals. Principals of schools in the centre of large cities were the least likely to report that shortages of teaching staff or instructional materials hinder learning, while principals in rural and remote communities were the most likely to report that such shortages hinder instruction. These differences closely mirror student PISA academic performance and school socioeconomic composition. PISA data indicates that schools located in small rural communities have the lowest socioeconomic profiles, the lowest academic performance and the largest shortages of teaching staff and instructional materials, while schools in central neighborhoods of large cities enjoy the highest socioeconomic profiles, the highest academic performance and the fewest shortages