bluuebunny/crossref_metadata_2025_split huggingface dataset:
PGAP2-Related Hyperphosphatasia-Mental Retardation Syndrome: Report of a Novel Patient, Toward a Broadening of Phenotypic Spectrum and Therapeutic Perspectives
Authors: Saracino Annalisa , Totaro Martina , Politano Davide , DE Giorgis Valentina , Gana Simone , Papalia Grazia , Pichiecchio Anna , Plumari Massimo , Rognone Elisa , Varesio Costanza , Orcesi Simona 
Published: February 2024

Abstract
PGAP2 gene has been known to be the cause of “hyperphosphatasia, mental retardation syndrome-3” (HPMRS3). To date, 14 pathogenic variants in PGAP2 have been identified as the cause of this syndrome in 24 patients described in single-case reports or small clinical series with pan-ethnic distribution. We aim to present a pediatric PGAP2-mutated case, intending to further expand the clinical phenotype of the syndrome and to report our experience on a therapeutic approach to drug-resistant epilepsy.We present the clinical, neuroradiological, and genetic characterization of a Caucasian pediatric subject with biallelic pathogenic variants in the PGAP2 gene revealed by next generation sequencing analysis.We identified a subject who presented with global developmental delay and visual impairment. Brain magnetic resonance imaging showed mild hypoplasia of the inferior cerebellar vermis and corpus callosum and mild white matter reduction. Laboratory investigations detected an increase in alkaline phosphatase. At the age of 13 months, he began to present epileptic focal seizures with impaired awareness, which did not respond to various antiseizure medications. Electroencephalogram (EEG) showed progressive background activity disorganization and multifocal epileptic abnormalities. Treatment with high-dose pyridoxine showed partial benefit, but the persistence of seizures and the lack of EEG amelioration prompted us to introduce ketogenic diet treatment.Our case provides a further phenotypical expansion of HPMRS3 to include developmental and epileptic encephalopathy. Due to the limited number of patients reported so far, the full delineation of the clinical spectrum of HPMRS3 and indications for precision medicine would benefit from the description of new cases and their follow-up evaluations.
Pericardial Decompression Syndrome: A Comprehensive Review of a Controversial Entity
Authors: Sarode Karan , Patel Amar , Arrington Kedzie , Makhija Rakhee , Mukherjee Debabrata 
Published: February 2024

AbstractPericardial decompression syndrome is an ambiguous clinical entity which has generated controversy regarding its existence. Following pericardial decompression, patients experienced clinical deterioration ranging in complications from pulmonary edema to death that could not be attributed to any other distinct clinical pathology. Multiple theories have suggested the pathophysiology behind pericardial decompression syndrome is related to preload-afterload mismatch following pericardial decompression, coronary microvascular ischemia, and stress from high adrenergic state. Our review aims to describe this syndrome by analyzing demographics, etiology of pericardial effusion, method of drainage, volume of pericardial fluid removed, time to decompensation, and clinical outcomes. A systematic review of MEDLINE/PubMed and Google Scholar literature databases were queried for case reports, case series, review articles, and abstracts published in English journals between 1983 and December 2022. Each author's interpretation of echocardiographic and/or pulmonary arterial catheterization data provided in the case reports was used to characterize ventricular dysfunction. Based on our inclusion criteria, 72 cases of pericardial decompression syndrome were included in our review. Our results showed that phenotypic heterogeneity was present based on echocardiographic findings of right/left or biventricular failure with similar proportions in each type of ventricular dysfunction. Time to decompensation was similar between immediate, subacute, and acute cases with presentation varying between hypoxic respiratory failure and shock. This review article highlights theories behind the pathophysiology, clinical outcomes, and therapeutic options in this high mortality condition.
Opportunity costs and global justice
Authors: Armstrong Chris 
Published: February 2024

Abstract
This chapter assesses how one kind of conservation burden—opportunity costs—should be understood and shows that our position on this issue has major implications for global justice. It goes on to argue that the notion of opportunity costs must be moralized, and then explains and defends an egalitarian baseline for calculating those costs. Taking this baseline seriously would suggest that many real-world conservation projects send far more modest resources in the direction of people affected by conservation projects than justice requires, and that they are often exploitative in nature. It examines the implications for conservation academics, practitioners, and policy makers.
Conclusion
Authors: Armstrong Chris 
Published: February 2024

Abstract
Vital efforts to tackle the biodiversity crisis can, if they are not designed carefully, produce considerable injustice. In the past, for example, conservation efforts have involved considerable political, epistemic, and distributive injustices. This conclusion draws together fifteen key arguments from the book, which highlight the perils of unjust conservation policies, and point the way forwards to just and inclusive alternatives. Having mapped the territory, it also aims to encourage others to develop their own visions of global justice as applied to the biodiversity crisis.
Philosophy for an Ending World
Authors: Mulgan Tim 
Published: February 2024

Abstract
This book introduces a new thought experiment—The Ending World—where the world will end in two hundred years, and humanity faces imminent and unavoidable (but not immediate) extinction. The book contains imaginary philosophical debates and lectures within this slowly ending world. The ending world is both a provocative thought experiment and a challenging possible future. Exploring it from within—adopting the perspective of philosophers living in that ending world—helps us to imagine this world from the inside, to evaluate it as a possible future, to discover what we owe to future people who might inhabit such a future, and to explore how we might justify ourselves to them. The book explores contemporary debates about pessimism, the meaning of life, the existence of God, the purpose of the universe, the permissibility of creating new people, the need to connect with past and future people, the rectification of historical injustice, the design of utopias, and the desirability of escaping into virtual realities. A central question throughout the book is whether we could equip our descendants to flourish in an ending world, even if we cannot imagine flourishing there ourselves. The book defends an innovative account of our obligations to future people, based on the need to launch multigenerational projects to transform our inherited traditions and values so that they will still make sense even at humanity’s end.
First Debate
Authors: Mulgan Tim 
Published: February 2024

Abstract
In this debate—set in an ending world where humanity faces unavoidable extinction in two hundred years—the Post-Cataclysm Pessimist argues that losing the indefinite human future is sufficient to make life meaningless. Samuel Scheffler has argued that even apparently present-focused projects presuppose an ongoing background of traditions, values, or relationships that extend into the future. The Post-Cataclysm Pessimist extends Scheffler’s critique, arguing that future-dependent meaning is lost even earlier, because our current (Pre-Cataclysm) values require an indefinite human future. As well as Scheffler, the debate draws on Jonathan Lear’s work on radical hope; nineteenth-century German pessimism, especially Eduard von Hartmann’s argument that meaning can only be found in future cultural progress; and Mark Johnston’s argument that good people literally ‘live on’ in future generations. The Post-Cataclysm Pessimist argues that all secular replacements for a personal afterlife collapse in the face of imminent extinction. The debate contrasts Post-Cataclysm pessimism with three main alternatives. The Perennial Pessimist argues that imminent extinction makes no difference, because life has always been meaningless. The Presentist argues that sufficient meaning can be found in present pleasures and achievements, or in care and compassion for the suffering of others. The Multigenerationalist replies that people living at the start of the ending world could raise new generations for whom meaning, well-being, and value are not dependent on the existence of any (further) future people.
Lecture Two
Authors: Mulgan Tim 
Published: February 2024

Abstract
This lecture—delivered in an ending world where humanity faces unavoidable extinction in two hundred years—introduces procreative ethics. People living at the start of a slowly ending world could create future people. But would this be permissible? In particular, is it permissible to create a last generation who cannot have children of their own? Most contemporary philosophical procreative ethicists share a broad commitment to procreative liberty. This lecture asks whether this commitment to procreative liberty could survive in a world facing imminent extinction, where it is challenged both by anti-natalists who insist that procreation is now forbidden and by ‘pro-natalists’ who argue that, in an uncertain world of impending extinction, procreative obligations are the only reliable way to ensure that enough people have children. The lecture summarises contemporary procreative ethics—covering Parfit’s non-identity problem, procreative asymmetry, and puzzles in aggregation such as Parfit’s repugnant conclusion. It then presents two particular approaches and applies them to the ending world: Elizabeth Harman’s harm-based approach and Melinda Roberts’s person-affecting consequentialism. Procreative ethics is vital to the case for multigenerationalism developed across all six lectures in this book. Multigenerationalism grounds present meaning in collaborations with future people. Therefore, multigenerational projects are permissible only if procreation is permissible. However, the lecture’s central arguments stand alone, and do not presuppose arguments made elsewhere in the book.
Lecture Five
Authors: Mulgan Tim 
Published: February 2024

Abstract
This lecture—delivered in an ending world where humanity faces unavoidable extinction in two hundred years—argues that the multigenerationalist approach introduced in Lecture One is feasible and distinctive. The lecture draws on Allen Buchanan and Russell Powell’s evolutionary defense of the possibility of moral progress; on Jonathan Lear’s philosophical exploration of the life of the Crow leader Plenty Coups who led his people into a future where their whole way of life no longer made sense; on recent work in environmental philosophy; and on Tim Mulgan’s earlier work on moral imaginativeness and possible futures. Against both progressive opponents who claim that future moral transformation is unnecessary, and conservative opponents who dismiss it as impossible, the lecture argues that multigenerationalism’s claims about moral progress, moral imaginativeness, and moral transformation are all credible—given our knowledge of human nature, and of past moral transformations. The lecture also argues that future moral transformation is necessary, not only for multigenerationalists, but for anyone who wants to permissibly create a new generation in a world where humanity faces unavoidable imminent extinction.
How and why to compare incomparables: The Great Gatsby and La Règle du jeu
Authors: Andrew Dudley 
Published: February 2024

Abstract
Comparing The Great Gatsby with any of its adaptations invariably promotes the novel over its derivatives and is far less fertile than putting it alongside Jean Renoir’s La Règle du jeu. During the interwar period, these incomparable masterpieces exhibit strikingly similar plots to address the moral decline of the upper class. Renoir directly borrowed this template from Musset’s 1833 Les Caprices de Marianne, a hallmark of Romanticism. The competing forces of romantic desire, aristocratic values, and aesthetic modernism make these works comparably difficult to classify. The article introduces Orson Welles’ Citizen Kane, which had already been compared to The Great Gatsby and was also filmed at the outset of WWII. Watching Welles’ film beside Renoir’s confirms André Bazin’s view that cinema became modern at just this moment, by taking on a role comparable to that commanded by post-WWI American novels like Fitzgerald’s. And examining Renoir beside Fitzgerald highlights the styles and strategies of both masterpieces as they pursued a large public and an authentic social critique. The article concludes by enumerating reasons and ways to compare ‘original’ works that bear similarities, whether mythic and universal or historical and particular.
Shared decision making in older people after severe stroke
Authors: Mead Gillian 
Published: February 2024

Abstract
Stroke is a major cause of death and lifelong disability. Although stroke treatments have improved, many patients are left with life-changing deficits. Shared decision making and consent are fundamental to good medical practice. This is challenging because stroke often causes mental incapacity, prior views might not be known and prognosis early after stroke is often uncertain. There are no large trials of shared decision making after severe stroke, so we need to rely on observational data to inform practice. Core ethical principles of autonomy, beneficence, non-maleficence and justice must underpin our decision making. ‘Surrogate’ decision makers will need to be involved if a patient lacks capacity, and prior expressed views and values and beliefs need to be taken into account in decision making. Patients and surrogates often feel shocked at the sudden nature of stroke, and experience grief including anticipatory grief. Health care professionals need to acknowledge these feelings and provide support, be clear about what decisions need to be made and provide sufficient information about the stroke, and the risks and benefits of treatments being considered. Shared decision making can be emotionally difficult for health care professionals and so working in a supportive environment with compassionate leadership is important. Further research is needed to better understand the nature of grief and what sort of psychological support would be most helpful. Large randomised trials of shared decision making are also needed.
Effect of Heart-Healthy Dietary Patterns on Blood Pressure: A Systematic Review and Meta-analysis
Authors: Jiao Xueyan , Song Kunpeng , Zhang Ning , Liu Luyao , Zhang Yifan , Chen Yun , Lan Zhenzhen , Liu Shutong , Liu Xincan 
Published: February 2024

Abstract

OBJECTIVE
To systematically evaluate the antihypertensive effect of dietary patterns of blood pressure management.

METHODS
The researchers searched the following databases: PubMed, Embase, the Cochrane Library, Web of Science, Scopus, China Knowledge Network, China Biomedical Literature Service, Wanfang Database, and Wipu.com by computer. Search for randomized controlled trials (RCTs) on the effects of Mediterranean diet, Dietary Approaches to Stop Hypertension (DASH), and Chinese Heart-Healthy (CHH) diet on blood pressure in English and Chinese. RCTs, which were published from the time of the study until 02/2023. The investigators assessed and screened the literature before conducting a systematic evaluation and meta-analysis using Stata 15 software.

RESULTS
This study included 27 publications containing 7,409 study subjects, including 3,677 in the trial group and 3,732 in the control group. Dietary models for blood pressure management were effective in reducing systolic blood pressure [weighted mean difference (WMD) = −3.94, 95% confidence interval (CI): (−5.10, −2.77), P &lt; 0.001] and diastolic blood pressure [WMD = −2.32, 95% CI: (−3.01, −1.63), P &lt; 0.001].

CONCLUSIONS
Dietary models for blood pressure management have a clear positive impact on blood pressure management in patients, and more high-quality studies with standardized design and long intervention period are needed in the future to provide strong evidence for healthy diet-based blood pressure management.
Relationship between publication of a postgraduate year 1 residency research project and subsequent career type at a large academic medical center
Authors: Nguyen Vi , Stevens Craig , Lepkowsky Marcie , Daniels Charles E , Patel Nimish 
Published: February 2024

Abstract

Purpose
The study objectives were to (1) quantify the overall incidence of residency publications of postgraduate year 1 (PGY1) residency alumni; (2) evaluate annual fluctuations in publications over time; and (3) compare the career types of residency alumni who published their PGY1 residency research projects to those for alumni who did not.

Methods
A retrospective cohort study was performed among individuals who completed a PGY1 acute/ambulatory care residency between 2010 and 2021. A list of residency alumni was obtained along with the corresponding titles of their research projects. Each resident’s name was entered into PubMed and Google Scholar to find the corresponding publication. LinkedIn and other publicly available resources were used to determine the career types of residents immediately after residency as well as their current career types.

Results
In total, 178 residency alumni completed an acute/ambulatory care PGY1 residency, of whom 16.7% (30/178) published their residency research project. Publication was associated with career type among those who pursued a postgraduate year 2 residency but was not associated with career type immediately after the PGY1 residency or current career type. The presence of an academic preceptor was associated with a higher probability of publishing compared to residents who did not have an academic preceptor (31.5% vs 10.5%; P &lt; 0.01).

Conclusion
The frequency of publications was within the range reported elsewhere, with fluctuations over time. Presence of an academic preceptor improved the probability of publication.
Influence of sleep quality on lapse to alcohol use during a quit attempt
Authors: Baskerville Wave-Ananda , Grodin Erica N , Ray Lara A 
Published: February 2024

Abstract

Aims
Sleep problems are common among individuals with alcohol use disorder (AUD) and is often associated with a heightened relapse risk. The present study examines the relationship between sleep and alcohol use among individuals with current AUD during a 6-day quit attempt as part of a medication study.

Methods
The current study is a secondary analysis of a medication trial for individuals with AUD. Individuals with AUD (N = 53, 26 females) were randomized to active medication or matched placebo. Randomized participants completed a week-long medication titration (Days 1–7). Following the titration period, participants attended an in-person visit (Day 8) to begin a 6-day quit attempt. During the quit attempt, participants completed daily diary assessments to report on previous day alcohol consumption, sleep quality, and alcohol craving. In the present study, medication condition was controlled for in all models.

Results
Baseline global sleep quality was not a significant predictor of drinks per drinking day (P = 0.72) or percent days abstinent (P = 0.16) during the 6-day practice quit attempt. Daily diary analyses found that greater sleep quality was associated with higher next-day drinks per drinking day (b = 0.198, P = 0.029). In contrast, participants reported worse sleep quality following nights of greater alcohol intake, albeit at a trend-level (b = −0.12, P = 0.053).

Conclusions
These results suggest that better sleep quality was a risk factor for drinking during the 6-day quit period, such that better sleep may be associated with increased craving for alcohol and alcohol use the next day. These findings are limited to the early abstinence period and should be considered in studies exploring longer periods of abstinence.
“Somewhere listening for my name”: Black Queer Kinship and the Poetry of the HIV/AIDS Pandemic
Authors: Cran Rona 
Published: February 2024

Abstract
This essay is about poetry, publication, and intergenerational caretaking in the context of a mass death event—the HIV/AIDS pandemic. It reads the work of contemporary Black, queer American poets Danez Smith, Jericho Brown, and Pamela Sneed for their intertextual and interpersonal engagement with queer and of-color literary texts and voices (in particular, those of Essex Hemphill, Melvin Dixon, and Donald Woods) under threat of erasure by HIV/AIDS and its effects and aftermaths. In doing so, it argues that Smith, Brown, and Sneed enact in their writing a political, spiritual, and historical project of recuperation and republication, taking the term “republishing” to encompass varying forms of print, performance, allusion, thematic evocation, formal echoes, and citation. In examining the complex, varied, and cross-temporal processes of poetic and scholarly caretaking and kinship—and of publication, “depublication,” and republication—this essay shows that the imprinting of HIV/AIDS into countercanonical poetry offers a crucial, ongoing, and collective counterweight to prevailing assumptions and stereotypes about the virus and the disease it causes, as well as creating and sustaining alternative sites of memory, mourning, and meaning making.The implications of the kind of republishing that Smith, Brown, and Sneed gesture toward—this way of remembering and reminding others of lost texts and writers—are manifold, if complex and unavoidably constrained, and include new readerships; the preservation of stories, legacies, and knowledge; and the mitigation of Black queer literary losses as a result of HIV/AIDS.
Queer Studies Now; or, The (Political) Economy, Stupid
Authors: Stein Jordan Alexander 
Published: February 2024

Abstract
The 1990s witnessed the origin of a decades-long “discipline problem” in queer studies, a conflict between empirical and theoretical approaches, and the two monographs under review—lesbian and gay historian Marc Stein’s Queer Public History: Essays on Scholarly Activism and queer theorist Lee Edelman’s Bad Education: Why Queer Theory Teaches Us Nothing—might seem like excellent candidates to reprise it. However, this review argues that the place where lesbian and gay history and queer theory have now caught up with one another is a structural one. What’s changed since the 1990s is less our disciplines and more the political–economic conditions that sustain them.What unites these two works in a common project, however, is the way each betrays an investment in the possibility—one might say, the hope—of political and social change.