Generalized,severe epidermolysis bullosa simplex caused by a Keratin 5 p.E477K mutation

Epidermolysis bullosa simplex (EBS) is a skin fragility disorder resulting from mutations of structural proteins in the epidermis. We provide a brief report of long‐term survival and reproduction in a mother with EBS due to keratin 5 (KRT5) c.1429G > A (p.E477K) mutation, which causes a particularly severe form of the disease.     

Multidimensionality of the PROMIS self-efficacy measure for managing chronic conditions

Quality of Life Research - This study investigated the PROMIS Self-Efficacy Measure for Managing Chronic Conditions (PROMIS-SE) domain distributions and examined the factor structure of the...     

Effects of Approach and Avoid Mindsets on Performance, Self-regulatory Cognition, and Affect in a Multi-task Environment

Despite numerous studies of approach- and avoidant mindsets, relatively little research has addressed the impact of such motivational orientations on performance and emotion in a real-time, multi-task setting. A laboratory simulation is reported that examines the influence of an induced approach-centered, an avoidance-centered, and a “neutral” motivational mindset upon multiple aspects of task performance, self-regulatory cognition, and affect. Undergraduate females randomly assigned to one of three mindset conditions performed a simulated automobile drive across one practice and two experimental trials. Dependent measures included divided attention, behavioral indicators of driving “cautiousness” in relatively safe straight roadway sections as well as during more risk-filled driving, multiple aspects of self-regulatory thinking (including self-monitoring, intended effort, and self-administered consequences), and positive and negative affect. Results revealed that the avoidant mindset produced poorer executive attention (i.e., fewer correctly detected divided attention events), more “cautious” driving behavior and reduced performance variability (i.e., greater control) when driving on presumably safe, straight roadway sections, lower self-reports of intended effort, and greater negative affect relative to the approach mindset. Results are intepreted within a self-regulation-centered motivational framework. Implications of the multi-task simulation for the study of normal and disordered adjustment are considered.