Genetic Counseling for Fanconi Anemia: Crosslinking Disciplines

Fanconi anemia (FA) is the most common of the inherited bone marrow failure syndromes with an incidence of approximately 1/100,000 to 1/200,000 live births. FA is a genetically complex and phenotypically heterogeneous condition involving birth defects, bone marrow failure, and cancer predisposition. This rare disease became well known in the genetic counseling community in 2002, when it was identified that biallelic mutations in BRCA2 can cause FA. Knowledge gained from the growing association between FA and breast cancer pathways has brought even more light to the complex genetic issues that arise when counseling families affected by this disease. Genetic counseling issues surrounding a diagnosis of FA affect many different disciplines. This review will serve as a way to cross-link the various topics important to genetic counselors that arise throughout the life of a patient with FA. Issues covered will include: an overview of FA, phenotypic presentation, management and treatment, the genetics and inheritance of FA, cytogenetic and molecular testing options, and the risks to family members of an individual with FA.     

The Importance of Pleasure in the Moral for Kant's Ethics

I argue for a new reading of Kant's claim that respect is the moral incentive; this reading accommodates the central insights of the affectivist and intellectualist readings of respect, while avoiding shortcomings of each. I show that within Kant's ethical system, the feeling of respect should be understood as paradigmatic of a kind of pleasure, pleasure in the moral. The motivational power of respect arises from its nature as pleasurable feeling, but the feeling does not directly motivate individual dutiful actions. Rather, the feeling is motivational in the sense that, after an agent has acted in a morally good way, the pleasure that results from that action contributes to the cultivation of virtue in the agent and, consequently, morally good actions in the future. Understanding the feeling of respect to be moral pleasure not only gives us insight into how finite rational beings develop virtue, but also a new way of understanding respect as an incentive.     

The World at 7:00: Comparing the Experience of Situations Across 20 Countries

The purpose of this research is to quantitatively compare everyday situational experience around the world. Local collaborators recruited 5,447 members of college communities in 20 countries, who provided data via a Web site in 14 languages. Using the 89 items of the Riverside Situational Q‐sort (RSQ), participants described the situation they experienced the previous evening at 7:00 p.m. Correlations among the average situational profiles of each country ranged from r = .73 to r = .95; the typical situation was described as largely pleasant. Most similar were the United States/Canada; least similar were South Korea/Denmark. Japan had the most homogenous situational experience; South Korea, the least. The 15 RSQ items varying the most across countries described relatively negative aspects of situational experience; the 15 least varying items were more positive. Further analyses correlated RSQ items with national scores on six value dimensions, the Big Five traits, economic output, and population. Individualism, Neuroticism, Openness, and Gross Domestic Product yielded more significant correlations than expected by chance. Psychological research traditionally has paid more attention to the assessment of persons than of situations, a discrepancy that extends to cross‐cultural psychology. The present study demonstrates how cultures vary in situational experience in psychologically meaningful ways.     

Efficacy of and preference for reinforcement and response cost in token economies

Researchers have shown that both differential reinforcement and response cost within token economies are similarly effective for changing the behavior of individuals in a group context (e.g., Donaldson, DeLeon, Fisher, & Kahng, 2014; Iwata & Bailey, 1974). In addition, these researchers have empirically evaluated preference for these procedures. However, few previous studies have evaluated the individual effects of these procedures both in group contexts and in the absence of peers. Therefore, we replicated and extended previous research by determining the individual effects and preferences of differential reinforcement and response cost under both group and individualized conditions. Results demonstrated that the procedures were equally effective for increasing on‐task behavior during group and individual instruction for most children, and preference varied across participants. In addition, results were consistent across participants who experienced the procedures in group and individualized settings.     

“Do identity and distinctness facts threaten the PSR?”

Philosophical Studies - One conception of the Principle of Sufficient Reason (PSR) maintains that every fact is metaphysically explained. There are different ways to challenge this version of the...     

Nostalgia motivates pursuit of important goals by increasing meaning in life

This research focused on existential and motivational implications of the emotion of nostalgia. Nostalgia (relative to control) increased meaning in life, which, in turn, galvanised intentions to pursue one's most important goal (Experiment 1) and to pursue one's most important, but not least important, goal (Experiment 2). The basic pattern held in two cultures (British and Danish) independently of positive affect. This is the first evidence that nostalgia has specific motivational consequences (i.e., pursuit of more, but not less, important goals) and transmits these consequences via meaning in life. Also, this is the first evidence that meaning is associated with specific motivational consequences. Discussion considers the relevance of the findings for the emotion and motivation literatures.     

Social Problems in ADHD: Is it a Skills Acquisition or Performance Problem?

Recent models suggest that social skills training’s inefficacy for children with ADHD may be due to target misspecification, such that their social problems reflect inconsistent performance rather than knowledge/skill gaps. No study to date, however, has disentangled social skills acquisition from performance deficits in children with ADHD. Children ages 8–12 with ADHD (n?=?47) and without ADHD (n?=?23) were assessed using the well-validated social behavioral analysis framework to quantify cross-informant social skills acquisition deficits, performance deficits, and strengths. Results provided support for the construct and predictive validities of this Social Skills Improvement System (SSIS) alternate scoring method, including expected magnitude and valence relations with BASC-2 social skills and ADHD symptoms based on both parent and teacher report. Acquisition deficits were relatively rare and idiosyncratic for both the ADHD and Non-ADHD groups, whereas children with ADHD demonstrated cross-informant social performance deficits (d?= 0.82–0.99) on several specific behaviors involving attention to peer directives, emotion regulation, and social reciprocity. Relative to themselves, children with ADHD were perceived by parents and teachers as exhibiting more social strengths than social acquisition deficits; however, they demonstrated significantly fewer social strengths than the Non-ADHD group (d?=??0.71 to ?0.89). These findings are consistent with recent conceptualizations suggesting that social problems in ADHD primarily reflect inconsistent performance rather than a lack of social knowledge/skills. Implications for refining social skills interventions for ADHD are discussed.     

Of Men and Money: Characteristics of Occupations that Affect the Gender Differentiation of Children’s Occupational Interests

Occupational interests become gender differentiated during childhood and remain so among adults. Two characteristics of occupations may contribute to this differentiation: the gender of individuals who typically perform the occupation (workers’ gender) and the particular goals that the occupation allows one to fulfill, such as the opportunity to help others or acquire power (value affordances). Two studies tested hypotheses about whether U.S. 6- to 11-year-olds show gender differences in their interest in novel jobs that were depicted as (a) being performed by men versus women and (b) affording money, power, family, or helping values. In Study 1, 98 children rank-ordered their preferences for experimentally-manipulated novel jobs, and they answered questions about their occupational values and the value affordances of jobs in which men and women typically work. In Study 2, a second sample of 65 children was used to test the replicability of findings from Study 1. As hypothesized, children were more interested in jobs depicted with same- than other-gender workers in both studies. Boys showed greater interest than did girls in novel jobs depicted as affording money in Study 1, but not Study 2. Explicit knowledge that men and women typically work in jobs that afford differing values increased with participants’ age.