Fabry Disease Practice Guidelines: Recommendations of the National Society of Genetic Counselors

Identification and comprehensive care of individuals who have Fabry disease (FD) requires a multidisciplinary approach inclusive of genetic testing, test interpretation, genetic counseling, long term disease symptom monitoring, treatment recommendations, and coordination of therapy. The purpose of this document is to provide health care professionals with guidelines for testing, care coordination, identification of psychosocial issues, and to facilitate a better understanding of disease treatment expert recommendations for patients with 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 representatives/founders of the two United States based Fabry disease patient advocacy groups who are themselves affected by Fabry disease. 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, interpretation of results, psychosocial considerations, and references to professional and patient resources.     

Bayesian tests of measurement invariance

Random item effects models provide a natural framework for the exploration of violations of measurement invariance without the need for anchor items. Within the random item effects modelling framework, Bayesian tests (Bayes factor, deviance information criterion) are proposed which enable multiple marginal invariance hypotheses to be tested simultaneously. The performance of the tests is evaluated with a simulation study which shows that the tests have high power and low Type I error rate. Data from the European Social Survey are used to test for measurement invariance of attitude towards immigrant items and to show that background information can be used to explain cross‐national variation in item functioning.     

Selection for Some,Facilitation for Others? Self-Control Theory and the Gang–Violence Relationship

Although self-control theory has been thought to be entirely consistent with the gang selection model, key theoretical predictions of the general theory imply gang selection effects for those with lower self-control and gang facilitation effects for those with higher self-control. This new hypothesis is tested among a large sample of jail inmates. Results indicate that self-control did not render the gang–violence relationship spurious for the sample as a whole. Gang membership had a significantly greater impact on violent crime among those with very high self-control, but there were still statistically significant gang facilitation effects for the other three self-control groups.     

Selective Processing of Threatening Words in Anxiety: The Role of Awareness

In three experiments, high and low trait-anxious individuals were required to classify a centrally located number as odd or even while ignoring spatially separate threat-related or neutral words. It was found that high anxious subjects showed a selective bias to process threatening stimuli when the stimuli were pattern masked after a brief exposure (14msec). However, this automatic processing bias was apparent only under certain conditions: distractors were within focal attention; masked and unmasked distractors were randomly intermixed; or masked trials were presented after unmasked trials. These results suggest that automatic processing biases in high traitanxious individuals are influenced by contextual factors. There was also a suggestion of a qualitative difference in attentional bias between conscious (unmasked) and nonconscious (masked) states, providing evidence for perception without awareness. The theoretical implications of these results are discussed.     

Affective primes suppress attention bias to threat in socially anxious individuals

Anxious individuals show an attention bias towards threatening information. However, under conditions of sustained environmental threat this otherwise-present attention bias disappears. It remains unclear whether this suppression of attention bias can be caused by a transient activation of the fear system. In the present experiment, high socially anxious and low socially anxious individuals (HSA group, n=12; LSA group, n=12) performed a modified dot-probe task in which they were shown either a neutral or socially threatening prime word prior to each trial. EEG was collected and ERP components to the prime and faces displays were computed. HSA individuals showed an attention bias to threat after a neutral prime, but no attention bias after a threatening prime, demonstrating that suppression of attention bias can occur after a transient activation of the fear system. LSA individuals showed an opposite pattern: no evidence of a bias to threat with neutral primes but induction of an attention bias to threat following threatening primes. ERP results suggested differential processing of the prime and faces displays by HSA and LSA individuals. However, no group by prime interaction was found for any of ERP components.     

Behavioral reactivity and approach-withdrawal bias in infancy

Seven hundred seventy-nine infants were screened at 4 months of age for motor and emotional reactivity. At age 9 months, infants who showed extreme patterns of motor and negative (n = 75) or motor and positive (n = 73) reactivity and an unselected control group (n = 86) were administered the Laboratory Temperament Assessment Battery, and baseline electroencephalogram data were collected. Negatively reactive infants showed significantly more avoidance than positively reactive infants and displayed a pattern of right frontal electroencephalogram asymmetry. Positively reactive infants exhibited significantly more approach behavior than controls and exhibited a pattern of left frontal asymmetry. Results support the notion that approach-withdrawal bias underlies reactivity in infancy.     

The Preliminary Employment Interview as a Predictor of Assessment Center Outcomes

The current study examined the relationships between personnel employment interview scores (PEI), cognitive ability test scores (CAT) and assessment center (AC) scores, as well as the potential to circumvent the costly AC method for some of the candidates by using these less expensive selection methods. A total of 423 Israeli police force candidates participated in the study. Their PEI and CAT scores were collected in the first stage of the selection process. They subsequently participated in an AC and a final decision was made regarding their acceptance to the police force. It was found that PEI and CAT scores significantly correlated with the AC scores and the recruitment decision, although the PEI scores demonstrated stronger correlations with the criteria. An actuarial analysis demonstrated the benefit of using the AC procedure for those achieving middle range scores on the PEI and CAT, circumventing the costly ACs for those achieving high and low scores. This strategy resulted in minor costs of both α and β errors. Organizations can adopt this economical strategy when the AC costs exceed the manpower costs incurred by disposing of the AC.