Fabry disease in genetic counseling practice: recommendations of the National Society of Genetic Counselors

The objective of this document is to provide health care professionals with recommendations for genetic counseling and testing of individuals with a suspected or confirmed diagnosis of Fabry disease, with a family history of Fabry disease, and those identified as female carriers of Fabry disease. These recommendations are the opinions of a multicenter working group of genetic counselors, medical geneticists, and other health professionals with expertise in Fabry disease counseling, as well as an individual with Fabry disease who is a founder of a Fabry disease patient advocacy group in the United States. The recommendations are U.S. Preventive Task Force Class III, and they are based on clinical experience, a review of pertinent English-language articles, and reports of expert committees. This document reviews the genetics of Fabry disease, the indications for genetic testing and interpretation of results, psychosocial considerations, and references for professional and patient resources. These recommendations should not be construed as dictating an exclusive course of management, nor does use of such recommendations guarantee a particular outcome. The professional judgment of a healthcare provider, familiar with the facts and circumstances of a specific case, will always supersede these recommendations.     

Helen Flanders Dunbar,John Dewey,and clinical pragmatism: reflections on method in psychosomatic medicine and bioethics

This article outlines the method utilized by physicians and major figures in the founding of Clinical Pastoral Education, Helen Flanders Dunbar, in her work of 1943, Psychosomatic Diagnosis, and relates it to the currently evolving approach in bioethics known as clinical pragmatism. It assesses Dewey's influence on both Dunbar in psychosomatic medicine and clinical pragmatism in bioethics, and illustrates the breadth of influence of the school of philosophical thought known as pragmatism with which Dewey's name and those of William James and Charles Sanders Pierce are most often identified.     

Personality, emotional experience, and efforts to control emotions

Three converging, multimethod studies examined personality and emotional processes. Study 1 (N = 321) examined links among sex, personality, and expectations for emotional events. In Study 2, participants (N = 468) described contents of emotionally evocative slides to a partner (either a friend or a stranger). Participants reported their emotional experience, efforts to control emotion, and the anticipated reactions of their partners. Structural modeling of self-report data and analyses of observational data indicated that Agreeableness and sex were significant predictors of emotional experience and of efforts to control emotion. Study 3 (N = 68) replicated and extended the two previous studies using psychophysiological methods to examine responses to positively and negatively charged emotional materials. Outcomes are discussed in terms of processes underlying the five-factor structural dimension of Agreeableness and links to emotional self-regulation.     

Punishments adolescents find justified: an examination of altitudes toward delinquency

The purpose of this study was to examine adolescents' attitudes toward delinquency, specifically the degree to which they find norm violations acceptable in various circumstances. A total of 1,914 Dutch youths participated: 1,046 reported having committed no crimes in the past year, 578 reported having committed only nonviolent offenses, and 290 reported having committed both violent and nonviolent offenses. It was hypothesized that, in general, delinquents would prescribe less severe punishments for deviant behavior, irrespective of the circumstances. This hypothesis was rejected. It was found, however, that violent delinquents were significantly more tolerant of violent behavior as compared with nondelinquents, with one important exception: when injury was inflicted on someone they knew, they were as little inclined to excuse the perpetrator as were nondelinquents. Violent delinquents discriminated most strongly between friends and strangers as both victims and perpetrators.     

Transfer of newly acquired stimulus valence between identities in dissociative identity disorder (DID)

Patients with Dissociative Identity Disorder (DID) frequently report episodes of interidentity amnesia, that is amnesia for events experienced by other identities. The goal of the present experiment was to test the implicit transfer of trauma-related information between identities in DID. We hypothesized that whereas declarative information may transfer from one identity to another, the emotional connotation of the memory may be dissociated, especially in the case of negative, trauma-related emotional valence. An evaluative conditioning procedure was combined with an affective priming procedure, both performed by different identities. In the evaluative conditioning procedure, previously neutral stimuli come to refer to a negative or positive connotation. The affective priming procedure was used to test the transfer of this acquired valence to an identity reporting interidentity amnesia. Results indicated activation of stimulus valence in the affective priming task, that is transfer of emotional material between identities.     

Dying to be thin: the effects of mortality salience and body mass index on restricted eating among women

Following terror management theory, the authors suggest women's striving to attain a thin physique is fueled in part by existential concerns. In three studies, women restricted consumption of a nutritious but fattening food in response to reminders of mortality (mortality salience; MS). When conducted in private (Study 1), this effect was found among women but not men; when replicated in a group setting in which social comparison was likely (Studies 2 and 3), only women who were relatively less successful attaining the thin ideal (i.e., high body mass index; BMI) restricted eating after MS. In Study 3, MS caused high BMI women to perceive themselves as more discrepant from their ideal thinness; this perceived failure mediated the effects of MS and BMI on eating behavior. Findings are discussed from a self-regulatory framework, which considered in the context of pressures for women to be thin, can shed light on health risk behavior.     

Empirical validation and psychometric evaluation of the Brief Fear of Negative Evaluation Scale in patients with social anxiety disorder

The Brief Fear of Negative Evaluation Scale (BFNE; M. R. Leary, 1983a) is often used to assess fear of negative evaluation, the core feature of social anxiety disorder. However, few studies have examined its psychometric properties in large samples of socially anxious patients. Although the BFNE yields a single total score, confirmatory factor analysis indicated a 2-factor solution to be more appropriate, with the 1st factor consisting of all straightforwardly worded items (BFNE-S) and the 2nd of all reverse-scored items (BFNE-R). Support was obtained for the convergent and discriminant validity of the BFNE and BFNE-S, but not the BFNE-R. These results suggest that standard scoring of the BFNE may not be optimal for patients with social anxiety disorder.     

Predictive validity of the Psychopathy Checklist: Youth Version for general and violent recidivism

Several authors have expressed concern regarding the use of youth psychopathy assessments in determinations of risk for general and violent offending. The Psychopathy Checklist: Youth Version (PCL:YV) was completed with 182 male adolescent offenders in this prospective study (average 14.5 month follow-up) of general and violent recidivism. Both a two-factor and three-factor model of the PCL:YV significantly predicted general and violent recidivism at a predictive accuracy ranging from 68 to 63%. However, regression analyses indicated these associations were explained primarily by behavioral psychopathic symptoms, rather than interpersonal or affective traits. Implications for the use of psychopathy assessments for risk during adolescence are discussed.     

Interidentity amnesia for neutral,episodic information in dissociative identity disorder

Interidentity amnesia is considered a hallmark of dissociative identity disorder (DID) in clinical practice. In this study, objective methods of testing episodic memory transfer between identities were used. Tests of both recall (interference paradigm) and recognition were used. A sample of 31 DID patients was included. Additionally, 50 control subjects participated, half functioning as normal controls and the other half simulating interidentity amnesia. Twenty-one patients subjectively reported complete one-way amnesia for the learning episode. However, objectively, neither recall nor recognition scores of patients were different from those of normal controls. It is suggested that clinical models of amnesia in DID may be specified to exclude episodic memory impairments for emotionally neutral material.