Correlation between neuroimaging, neurological phenotype, and functional outcomes in Wilson's disease.

INTRODUCTION: Wilson's disease (WD) is associated with a variety of movement disorders and progressive neurological dysfunction. The aim of this study was to correlate baseline brain magnetic resonance imaging (MRI) features with clinical phenotype and long-term outcomes in chronically treated WD patients. METHODS: Patients were retrospectively selected from an institutional database. Two experienced neuroradiologists reviewed baseline brain MRI. Functional assessment was performed using the World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0) scale, and disease severity was classified using the Global Assessment Scale for Wilson's Disease (GASWD). RESULTS: Of 27 patients selected, 14 were female (51.9%), with a mean (standard deviation [SD]) age at onset of 19.5 (7.1) years. Neurological symptoms developed in 22 patients (81.5%), with hyperkinetic symptoms being the most common (70.4%). Baseline brain MRI showed abnormal findings in 18 cases (66.7%), including T2 hyperintensities in 59.3% and atrophy in 29.6%. After a mean (SD) follow-up of 20.9 (11.0) years, WD patients had a mean score of 19.2 (10.2) on WHODAS 2.0 and 6.4 (5.7) on GASWD. The presence of hyperkinetic symptoms correlated with putaminal T2 hyperintensities (p = 0.003), putaminal T2 hypointensities (p = 0.009), and mesencephalic T2 hyperintensities (p = 0.009). Increased functional disability was associated with brain atrophy (p = 0.007), diffusion abnormalities (p = 0.013), and burden of T2 hyperintensities (p = 0.002). A stepwise regression model identified atrophy as a predictor of increased WHODAS 2.0 (p = 0.023) and GASWD (p = 0.007) scores. CONCLUSIONS: Atrophy and, to a lesser extent, deep T2 hyperintensity are associated with functional disability and disease severity in long-term follow-up of WD patients.

B-cell compartment abnormalities are associated with ACLF and mortality in patients with liver cirrhosis.

BACKGROUND: Liver cirrhosis profoundly affects the immune system, leading to an immunological imbalance known as cirrhosis-associated immune dysfunction. AIMS: This study aimed to investigate B-cell disturbances in patients with acute decompensation (AD) of cirrhosis and assess relationships with prognosis and mortality. METHODS: The study included 39 patients with AD of cirrhosis, 29 patients with stable cirrhosis (SC), and 30 healthy controls (CTR). Circulating B-cell subsets and cytokine plasma levels were determined by flow cytometry. RESULTS: Cirrhotic groups showed higher percentages of naïve B cells, and lower percentages of CD27+ memory B cells (MBCs) than CTR. Further analysis comparing SC and AD revealed that the latter had higher frequencies of double-negative (DN) B cells and plasmablasts. Patients with more advanced liver disease exhibited a B-cell maturation shift toward MBCs and plasmablasts. Acute-on-chronic liver failure (ACLF) was associated with higher DN frequency. The Kaplan-Meier one-year survival probability was 92.9% in patients with >1.3% of transitional B cells and 27.3% in patients with <1.3%. CONCLUSIONS: B-cell subsets are markedly altered in cirrhotic patients, and cell profiles differ between stable and decompensated liver disease. Increased frequencies of DN B cells and reduced proportions of transitional B cells may be of great relevance in predicting ACLF and mortality, respectively.

Patterns of dendritic cell and monocyte subsets are associated with disease severity and mortality in liver cirrhosis patients.

Liver cirrhosis is often complicated by an immunological imbalance known as cirrhosis-associated immune dysfunction. This study aimed to investigate disturbances in circulating monocytes and dendritic cells in patients with acute decompensation (AD) of cirrhosis. The sample included 39 adult cirrhotic patients hospitalized for AD, 29 patients with stable cirrhosis (SC), and 30 healthy controls (CTR). Flow cytometry was used to analyze monocyte and dendritic cell subsets in whole blood and quantify cytokines in plasma samples. Cirrhotic groups showed higher frequencies of intermediate monocytes (iMo) than CTR. AD patients had lower percentages of nonclassical monocytes than CTR and SC. Cirrhotic patients had a profound reduction in absolute and relative dendritic cell numbers compared with CTR and showed higher plasmacytoid/classical dendritic cell ratios. Increased plasma levels of IL-6, IL-10, and IL-17A, elevated percentages of CD62L+ monocytes, and reduced HLA-DR expression on classical monocytes (cMo) were also observed in cirrhotic patients. Patients with more advanced liver disease showed increased cMo and reduced tissue macrophages (TiMas) frequencies. It was found that cMo percentages greater than 90.0% within the monocyte compartment and iMo and TiMas percentages lower than 5.7% and 8.6%, respectively, were associated with increased 90-day mortality. Monocytes and dendritic cells are deeply altered in cirrhotic patients, and subset profiles differ between stable and advanced liver disease. High cMo and low TiMas frequencies may be useful biomarkers of disease severity and mortality in liver cirrhosis.

Belief in a just world and self-efficacy to promote justice in the world predict helping attitudes, but only among volunteers

Previous studies have shown that willingness to help particular victims is predicted separately, or in combination, by the belief in a just world (BJW) and self-efficacy to promote justice in the world (SEJW). Our goal was to extend this knowledge by studying general attitudes towards helping and the association between BJW and SEJW in people that do volunteer service, and by comparing the predictive power of two spheres of BJW, personal BJW and general BJW, on helping attitudes. We measured personal BJW, general BJW, SEJW and helping attitudes in two samples (one comprising university students and the other only volunteers). The results differed in the two samples. For volunteers endorsing strong self-efficacy to promote justice in the world, personal BJW was associated positively with helping attitudes (p = .007). For participants endorsing weak self-efficacy to promote justice in the world, personal BJW was not associated with helping attitudes in either sample. General BJW was not associated with helping attitudes in either sample, either independently or in interaction with self-efficacy to promote justice in the world. The theoretical and practical implications of these results are discussed (AU)

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Perceptions of the self and most people's reactions towards innocent and noninnocent victims.

Research has shown that: individuals positively distinguish themselves from most other people; being consistent is positively valued; injunctive and descriptive norms are perceived to protect victims. Joining these findings, we argue that individuals present themselves as following injunctive and descriptive norms towards victims to a higher extent and more consistently than most people. In an experimental study 273 university students of both sexes indicated what they and most other people would approve of (injunctive norm) or typically do (descriptive norm) regarding various reactions towards either an innocent or a noninnocent victim. The reactions involved secondary victimization (devaluation/derogation, avoidance, suffering minimization, blaming the victim) and non secondary victimization (valuation, contact, suffering acknowledgment, not blaming the victim). Participants perceived themselves and most people as approving of more non secondary than secondary victimization reactions, except for blaming the noninnocent victim. Participants indicated they approved of most of the normative reactions to a higher extent than most people, which is interpreted as a new instance of the Primus Inter Pares effect. Participants also indicated they would show more consistency between their injunctive and descriptive norms, especially towards the innocent victim. Results suggest that individuals perceive themselves as more immune to perverse norms than most people.

Perceptions of the self and most people’s reactions towards innocent and non innocent victims

Research has shown that: individuals positively distinguish themselves from most other people; being consistent is positively valued; injunctive and descriptive norms are perceived to protect victims. Joining these findings, we argue that individuals present themselves as following injunctive and descriptive norms towards victims to a higher extent and more consistently than most people. In an experimental study 273 university students of both sexes indicated what they and most other people would approve of (injunctive norm) or typically do (descriptive norm) regarding various reactions towards either an innocent or a noninnocent victim. The reactions involved secondary victimization (devaluation/ derogation, avoidance, suffering minimization, blaming the victim) and non secondary victimization (valuation, contact, suffering acknowledgment, not blaming the victim). Participants perceived themselves and most people as approving of more non secondary than secondary victimization reactions, except for blaming the noninnocent victim. Participants indicated they approved of most of the normative reactions to a higher extent than most people, which is interpreted as a new instance of the Primus Inter Pares effect. Participants also indicated they would show more consistency between their injunctive and descriptive norms, especially towards the innocent victim. Results suggest that individuals perceive themselves as more immune to perverse norms than most people (AU)

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Personal and general belief in a just world as judgement norms.

Research on the belief in a just world (BJW) has focused on its intra-individual functions (e.g., psychological well-being) and its inter-individual consequences (e.g., derogation of victims). Recent theorizing, however, has indicated that the BJW may also have more societal functions and consequences, serving as a legitimizing device of the status quo. The studies in the current paper focus on this latter view and are based on Alves and Correia's ( 2008 ) research which found that the expression of high personal and general belief in a just world is injunctively normative. Two experimental studies aimed at ascertaining three issues: (1) the dimension(s) of social value (social utility and/or social desirability) on which the BJW normativity anchors; (2) whether the expression of moderate BJW is also injunctively normative; and (3) whether the injunctive normativity of the BJW is related to perceptions of truth. Results indicate that moderate and high personal and general BJW are normative. Yet, whereas the normativity of personal BJW anchors both on social utility and social desirability, that of general BJW anchors only on social utility. We discuss personal and general BJW as judgement norms, whose normativity may not be personally acknowledged (in the case of general BJW) and does not necessarily derive from being perceived as true, but from the fact that such norms carry social value at least in individualistic societies.

Norms regarding secondary victimization of bullying victims: do they differ according to the victim's categorization?

Two studies with a 2 valence of the image (positive, negative) x 2 victim's category (ingroup; outgroup) between-subjects design, investigated the existence of prescriptive norms regarding secondary victimization of bullying victims as well as the influence of the categorization of the victim on those same prescriptive norms among 7th graders. Study 1 addressed a scenario of physical bullying. Study 2 addressed a scenario of verbal bullying combined with social exclusion. Results showed that norms prescribe that the ingroup victim of physical bullying should be the least secondarily victimized. Furthermore it was found that for both types of bullying a positive image can be conveyed by secondarily victimizing more an outgroup victim than an ingroup victim.