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In this study, we expanded upon the job demands-resources model to assess the role of employees' vitality as an inner resource for their work engagement and job commitment. To assess vitality and related job resources, we developed an index of vitality outside of work and adapted measures of manager autonomy support and organizational support. For job demands, we measured work stress and predicted that each of these four variables would contribute independently to work-related outcomes. Then, in a preregistered study, we collected these measures from a sample of 5,280 American workers (primarily ages 18-34, 54% female). Results from multivariate regression analyses largely confirmed our hypotheses, showing that positive work-related outcomes, such as enthusiasm, enjoyment, and job satisfaction, were positively predicted by manager autonomy supports, organizational support, and individuals' vitality, and negatively predicted by work stress. The reverse pattern was largely observed for the negative outcome of turnover intention. Exploratory analyses also suggested that individual vitality may buffer the negative effects of stress and low manager and organizational support. The results highlight the potential role of employee vitality outside of work and managerial support in bolstering work engagement and reducing turnover intentions, offering a basis for organizational strategies aimed at improving work culture and retaining talent., Competing Interests: CD, CR, and RR were employed by Immersyve, Inc. SD-C and TT were employed by Evernorth Research Institute. The remaining author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 DeHaan, Bradshaw, Diaz-Castillo, Trautman, Rigby and Ryan.)

Introduction: Pathogenic variants in STXBP1 cause a spectrum of disorders mainly consisting of developmental and epileptic encephalopathy (DEE), often featuring drug-resistant epilepsy. An increased mortality risk occurs in individuals with drug-resistant epilepsy and DEE, with sudden unexpected death in epilepsy (SUDEP) often the major cause of death. This study aimed to identify the rate and causes of mortality in STXBP1-related disorders., Methods: Through an international call, we analyzed data on individuals with STXBP1 pathogenic variants, who passed away from causes related to their disease., Results: We estimated a mortality rate of 3.2% (31/966), based on the STXBP1 Foundation and the STXBP1 Global Connect registry. In total, we analyzed data on 40 individuals (23 males) harboring pathogenic STXBP1 variants, collected from different centers worldwide. They died at a median age of 13 years (range: 11 months-46 years). The most common cause of death was SUDEP (36%), followed by pulmonary infections and respiratory complications (33%). The incidence of SUDEP peaked in mid-childhood, while non-SUDEP causes were more frequent in early childhood or adulthood (p = 0.006). In the most severe phenotypes, death was related to non-SUDEP causes (p = 0.018)., Conclusion: We found a mortality rate in STXBP1-related disorders similar to other DEEs, with an early age at death and SUDEP as well as pulmonary infections as the main cause of death. These findings assist in prognostic evaluation and genetic counseling for the families. They help to define the mortality risk of STXBP1-related disorders and implement preventative strategies., (© 2024. The Author(s).)

Description Research shows that when educational leaders support their learners' autonomy, it positively impacts both parties. This is particularly important in graduate medical education (GME), given that there is a strong emphasis on resident performance, evaluation, and development. Unfortunately, GME faculty often misunderstand autonomy as the resident's desire for independence or "freedom," when in fact it refers to the core psychological need to feel volitional and agentic. The distinction is important because volition is not synonymous with independence, and providing freedom can be at odds with strategies that provide true autonomy support. This, in turn, can contribute to the stress, maladjustment, and resident burnout that are already prevalent in medicine. To help remedy this issue, this paper provides an evidence-based guide for medical educators to distinguish autonomy from independence, with specific examples to help translate theory into practice to better support the well-being of the medical community., Competing Interests: Conflicts of Interest: The authors report honoraria and financial support from HCA Healthcare Graduate Medical Education., (© 2024 HCA Physician Services, Inc. d/b/a Emerald Medical Education.)

Objective: Individuals with disease-causing variants in STXBP1 frequently have epilepsy onset in the first year of life with a variety of seizure types, including epileptic spasms. However, the impact of early onset seizures and antiseizure medication (ASM) on the risk of developing epileptic spasms and impact on their trajectory are poorly understood, limiting informed and anticipatory treatment, as well as trial design., Methods: We retrospectively reconstructed seizure and medication histories in weekly intervals for individuals with STXBP1 developmental and epileptic encephalopathy (DEE) with epilepsy onset in the first year of life and quantitatively analyzed longitudinal seizure histories and medication response., Results: We included 61 individuals with early onset seizures, 29 of whom had epileptic spasms. Individuals with neonatal seizures were likely to have continued seizures after the neonatal period (25/26). The risk of developing epileptic spasms was not increased in individuals with neonatal seizures or early infantile seizures (21/41 vs. 8/16, odds ratio [OR] = 1, 95% confidence interval [CI] = .3-3.9, p = 1). We did not find any ASM associated with the development of epileptic spasms following prior seizures. Individuals with prior seizures (n = 16/21, 76%) had a higher risk of developing refractory epileptic spasms (n = 5/8, 63%, OR = 1.9, 95% CI = .2-14.6, p = .6). Individuals with refractory epileptic spasms had a later onset of epileptic spasms (n = 20, median = 20 weeks) compared to individuals with nonrefractory epileptic spasms (n = 8, median = 13 weeks, p = .08)., Significance: We provide a comprehensive assessment of early onset seizures in STXBP1-DEE and show that the risk of epileptic spasms is not increased following a prior history of early life seizures, nor by certain ASMs. Our study provides baseline information for targeted treatment and prognostication in early life seizures in STXBP1-DEE., (© 2024 International League Against Epilepsy.)

STXBP1-related disorders are among the most common genetic epilepsies and neurodevelopmental disorders. However, the longitudinal epilepsy course and developmental end points, have not yet been described in detail, which is a critical prerequisite for clinical trial readiness. Here, we assessed 1281 cumulative patient-years of seizure and developmental histories in 162 individuals with STXBP1-related disorders and established a natural history framework. STXBP1-related disorders are characterized by a dynamic pattern of seizures in the first year of life and high variability in neurodevelopmental trajectories in early childhood. Epilepsy onset differed across seizure types, with 90% cumulative onset for infantile spasms by 6 months and focal-onset seizures by 27 months of life. Epilepsy histories diverged between variant subgroups in the first 2 years of life, when individuals with protein-truncating variants and deletions in STXBP1 (n = 39) were more likely to have infantile spasms between 5 and 6 months followed by seizure remission, while individuals with missense variants (n = 30) had an increased risk for focal seizures and ongoing seizures after the first year. Developmental outcomes were mapped using milestone acquisition data in addition to standardized assessments including the Gross Motor Function Measure-66 Item Set and the Grasping and Visual-Motor Integration subsets of the Peabody Developmental Motor Scales. Quantification of end points revealed high variability during the first 5 years of life, with emerging stratification between clinical subgroups. An earlier epilepsy onset was associated with lower developmental abilities, most prominently when assessing gross motor development and expressive communication. We found that individuals with neonatal seizures or early infantile seizures followed by seizure offset by 12 months of life had more predictable seizure trajectories in early to late childhood compared to individuals with more severe seizure presentations, including individuals with refractory epilepsy throughout the first year. Characterization of anti-seizure medication response revealed age-dependent response over time, with phenobarbital, levetiracetam, topiramate and adrenocorticotropic hormone effective in reducing seizures in the first year of life, while clobazam and the ketogenic diet were effective in long-term seizure management. Virtual clinical trials using seizure frequency as the primary outcome resulted in wide range of trial success probabilities across the age span, with the highest probability in early childhood between 1 year and 3.5 years. In summary, we delineated epilepsy and developmental trajectories in STXBP1-related disorders using standardized measures, providing a foundation to interpret future therapeutic strategies and inform rational trial design., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Guarantors of Brain.)

Objective: STXBP1-related disorders are rare genetic epilepsies and neurodevelopmental disorders, but the impact of symptoms across clinical domains is poorly understood. Disease concept models are formal frameworks to assess the lived experience of individuals and their families and provide a basis for generating outcome measures., Methods: We conducted semistructured, qualitative interviews with 19 caregivers of 16 individuals with STXBP1-related disorders and 7 healthcare professionals. We systematically coded themes using NVivo software and grouped concepts into the domains of symptoms, symptom impact, and caregiver impact. We quantified the frequency of concepts throughout the lifespan and across clinical subgroups stratified by seizure history and developmental trajectories., Results: Over 25 hours of interviews, we coded a total of 3626 references to 38 distinct concepts. In addition to well-recognized clinical features such as developmental delay (n = 240 references), behavior (n = 201), and seizures (n = 147), we identified previously underrepresented symptoms including gastrointestinal (n = 68) and respiratory symptoms (n = 24) and pain (n = 30). The most frequently referenced symptom impacts were autonomy (n = 96), socialization (n = 64), and schooling (n = 61). Emotional impact (n = 354), support (n = 200), and daily life & activities (n = 108) were highly cited caregiver impacts. We found that seizures were more commonly referenced in infancy than in other age groups, while behavior and socialization were more likely to be referred to in childhood. We found that caregivers of individuals with ongoing seizures were less likely to reference developmental delay, possibly due to the relatively high impact of seizures., Significance: STXBP1-related disorders are complex conditions affecting a wide range of clinical and social domains. We comprehensively mapped symptoms and their impact on families to generate a comprehensive disease model as a foundation for clinical endpoints in future trials., (© 2023 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

While many genetic diseases have effective treatments, they frequently progress rapidly to severe morbidity or mortality if those treatments are not implemented immediately. Since front-line physicians frequently lack familiarity with these diseases, timely molecular diagnosis may not improve outcomes. Herein we describe Genome-to-Treatment, an automated, virtual system for genetic disease diagnosis and acute management guidance. Diagnosis is achieved in 13.5 h by expedited whole genome sequencing, with superior analytic performance for structural and copy number variants. An expert panel adjudicated the indications, contraindications, efficacy, and evidence-of-efficacy of 9911 drug, device, dietary, and surgical interventions for 563 severe, childhood, genetic diseases. The 421 (75%) diseases and 1527 (15%) effective interventions retained are integrated with 13 genetic disease information resources and appended to diagnostic reports ( https://gtrx.radygenomiclab.com ). This system provided correct diagnoses in four retrospectively and two prospectively tested infants. The Genome-to-Treatment system facilitates optimal outcomes in children with rapidly progressive genetic diseases., (© 2022. The Author(s).)

Health avatars were created to deliver previously tested live interventions for tobacco dependence and cholesterol management. The exploratory aims were to develop and test whether the avatar can be reliably assessed for autonomy supportiveness using the Health Care Climate Questionnaire and estimate the mean changes in motivation variables and correlate the avatars' autonomy supportiveness with the motivation variables and health outcomes. The avatars were found to be reliably assessed for autonomy supportiveness on the Health Care Climate Questionnaire. Autonomy support was positively correlated with the change in motivations and reduction in low-density lipoprotein. These findings suggest that health avatars may be tested in clinical trials.

There is a lack of research on the use of electronic tools that guide patients toward reducing their cardiovascular disease risk. We conducted a 9-month clinical trial in which participants who were at low (n = 100) and moderate (n = 23) cardiovascular disease risk-based on the National Cholesterol Education Program III's 10-year risk estimator-were randomized to usual care or to usual care plus use of an Interactive Cholesterol Advisory Tool during the first 8 weeks of the study. In the moderate-risk category, an interaction between treatment condition and Framingham risk estimate on low-density lipoprotein and non-high-density lipoprotein cholesterol was observed, such that participants in the virtual clinician treatment condition had a larger reduction in low-density lipoprotein and non-high-density lipoprotein cholesterol as their Framingham risk estimate increased. Perceptions of the Interactive Cholesterol Advisory Tool were positive. Evidence-based information about cardiovascular disease risk and its management was accessible to participants without major technical challenges., Competing Interests: Declaration of Conflicting Interest: Geoffrey C. Williams and C. Scott Rigby are shareholders in Self-Determined Health, Inc., the company to which the SBIR grant was awarded. Drs. Williams and Rigby were involved in the development of the virtual clinician and contributed only to the introduction and methods sections of this report. Dr. Williams managed subjects in both of the moderate risk groups, and those subjects were informed (via the study consent form) of Dr. Williams’ interest in the company. Drs. Williams and Rigby did not have access to the data or contribute to the interpretation of the results., (© The Author(s) 2015.)

[Correction Notice: An Erratum for this article was reported in Vol 106(3) of Journal of Personality and Social Psychology (see record 2014-07574-006). In the article, the name of author Edward Deci was missing his middle name initial and should have read as Edward L. Deci. In addition, an incorrect version of figure 1 was published.] Recent studies have examined whether electronic games foster aggression. At present, the extent to which games contribute to aggression and the mechanisms through which such links may exist are hotly debated points. In current research we tested a motivational hypothesis derived from self-determination theory-that gaming would be associated with indicators of human aggression to the degree that the interactive elements of games serve to impede players' fundamental psychological need for competence. Seven studies, using multiple methods to manipulate player competence and a range of approaches for evaluating aggression, indicated that competence-impeding play led to higher levels of aggressive feelings, easier access to aggressive thoughts, and a greater likelihood of enacting aggressive behavior. Results indicated that player perceived competence was positively related to gaming motivation, a factor that was, in turn, negatively associated with player aggression. Overall, this pattern of effects was found to be independent of the presence or absence of violent game contents. We discuss the results in respect to research focused on psychological need frustration and satisfaction and as they regard gaming-related aggression literature., ((PsycINFO Database Record (c) 2014 APA, all rights reserved).)

The present research examined the background and consequences of different styles of engagement in video game play. Based on self-determination theory(1) and the dualistic model of passion,(2) the authors hypothesized that high levels of basic psychological need satisfaction would foster harmonious passion for video play, supporting the subjective sense that play is something one wants to do. It was also predicted that low levels of need satisfaction would promote obsessive passion for games and contribute to the feeling that game play is something one feels compelled to or has to do. It was expected, in turn, that passion for play would directly influence player outcomes closely tied to games, moderate links between play and well-being, and relate to overall levels of well-being as a function of basic need satisfaction. As expected, results showed that low levels of basic need satisfaction were associated with more obsessive passion, higher amounts of play, greater tension following play, and low game enjoyment, whereas high levels of need satisfaction did not predict hours of play but were associated with more harmonious passion, game enjoyment, and energy following play. Moderation analyses showed that high amounts of play related negatively to well-being only to the extent that players reported an obsessive passion and that the unique relations between passion and overall levels of player well-being were quite small once controlling for their basic need satisfaction in daily life. Discussion of the current findings focuses on their significance for understanding disordered play and the value of applying a theory-based approach to study motivation for virtual contexts.

Six studies, two survey based and four experimental, explored the relations between violent content and people's motivation and enjoyment of video game play. Based on self-determination theory, the authors hypothesized that violence adds little to enjoyment or motivation for typical players once autonomy and competence need satisfactions are considered. As predicted, results from all studies showed that enjoyment, value, and desire for future play were robustly associated with the experience of autonomy and competence in gameplay. Violent content added little unique variance in accounting for these outcomes and was also largely unrelated to need satisfactions. The studies also showed that players high in trait aggression were more likely to prefer or value games with violent contents, even though violent contents did not reliably enhance their game enjoyment or immersion. Discussion focuses on the significance of the current findings for individuals and the understanding of motivation in virtual environments.

Adult respiratory distress syndrome occurs concomitantly with a number of clinical conditions but has no known cause. At present, there is no generally acceptable method for establishing the early diagnosis. In the course of studying immune aberrations by means of monoclonal antibody staining and fluorescence-activated cell sorting in injured patients, we noted an apparent specific T-lymphocyte response to this syndrome in one patient. There was an increase in suppressor T-cells and a decrease in helper/suppressor T-lymphocyte ratio (normal = 1.57). When the patient recovered, the helper/suppressor ratio rose to above 3.0. T-lymphocyte analysis offers a promising means of evaluating patients considered highly susceptible to adult respiratory distress syndrome, i.e., victims of massive trauma. Further studies will be required to fully elucidate this possibility.