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Experts frequently assess competency in criminal settings where the rate of feigning cognitive deficit is demonstrably elevated. We describe the construction and validation of the Denney Competency Related Test (D-CRT) to assess feigned incompetency of defendants in the criminal adjudicative setting. It was expected the D-CRT would prove effective at identifying feigned incompetence based on its two alternative, forced-choice and performance curve characteristics. Development and validation of the D-CRT occurred in described phases. Items were developed to measure competency based upon expert review. Item analysis and adjustments were completed with 304 young teenage volunteers to obtain a proper spread of item difficulty in preparation for eventual performance curve analysis (PCA). Test-retest reliability was assessed with 44 adult community volunteers. Validation included an analog simulation design with 101 jail detainees using MacArthur Competency Assessment Test-Criminal Adjudication and Word Memory Test as criterion measures. Effects of racial/ethnic demographic differences were examined in a separate study of 208 undergraduate volunteers. D-CRT specificity was identified with 46 elderly clinic referrals diagnosed with mild cognitive impairment and dementia. Item development, adjustment, and repeat analysis resulted in item probabilities evenly spread from.28 to 1.0. Test-retest correlation was good (.83). Internal consistency of items was excellent (KR-20 >.91). D-CRT demonstrated convergent validity in regard to measuring competency related information and as well as malingering. The test successfully differentiated between jail inmates asked to perforfm their best and inmates asked to simulate incompetency (AUC =.945). There were no statistically significant differences found in performance across racial/ethnic backgrounds. D-CRT specificity remained excellent among elderly clinic referrals with significant cognitive compromise at the recommended total score cutoff. D-CRT is an effective measure of feigned criminal incompetency in the context of potential cognitive deficiency, and PCA is assistive in the determination. Additional validation using knowns groups designs with various mental health-related conditions are needed. [ABSTRACT FROM AUTHOR]

Glycogen storage disease (GSD) type 1a is an inherited autosomal recessive metabolic disease caused by a deficiency in glucose-6-phosphatase activity. The objectives of this research were to systematically review the published literature on the epidemiology of GSD 1a and to assess the performance of reported epidemiology measures in a simulation model. In this systematic literature review 2,539 record titles and abstracts were screened. Of these, only 11 studies contained relevant data on GSD 1a disease epidemiology. Reported disease frequency ranged from 0.085/100,000 to 10.3/100,000 newborns when considering all the GSD literature. When this was narrowed to GSD 1 and GSD 1a, the range was tightened to 0.25–3.02/100,000 and 0.085–4.9/100,000 newborns, respectively. Most of the identified studies counted the number of diagnoses in a defined period and related to the number of births in the same (Dx method) or different time period (DoB method). The simulation model results indicate that in most of the situations, the Dx method provides a closer estimate to the true disease incidence than the DoB method. Despite the scarcity of epidemiology data, the results of this systematic review strongly support that GSD 1a and its parent disease groups (GSD and GSD 1) are rare diseases. [ABSTRACT FROM AUTHOR]

Stroke significantly impairs health-related quality of life (HRQL). Stroke survivors with aphasia (SWA) experience lower HRQL than stroke survivors without aphasia (SSA) as a result of poorer communication and social functioning. The extent to which aphasia influences HRQL in African-Americans and the components of social functioning that are most important to HRQL warrants further exploration. There were two main objectives of this paper. The first was to survey HRQL domains of communication, physical, mental/emotional, role, and social functioning in African American SWA and SSA. The second was to examine if social support and social network predicted HRQL in SWA. A total of 39 African American adults (62.4 ± 11.10) participated in this descriptive cross-sectional case control study. Patient-reported outcome measures were used to assess HRQL, perceived social support, and social network in SWA, SSA, and normal-aging healthy controls (NAH). Data analysis included an ANOVA and moderator regression to determine if social support or social network predicted HRQL in SWA. SWA reported a significantly lower overall HRQL (p = <.000) than NAH adults. Communication HRQL was the hallmark difference found between SWA and SSA (p = <.000). Social support and social network were relatively similar among all three groups. However, social support and social network did not predict HRQL in SWA. Findings from this study suggest that social HRQL continues to be significantly lower in SWA; however, social support and social network factors do not drive differences among African-Americans. Moreover, communication HRQL remains the hallmark difference between SWA and SSA. [ABSTRACT FROM AUTHOR]

Background: In academic research and the pharmaceutical industry, in vitro cell lines and in vivo animal models are considered as gold standards in modelling diseases and assessing therapeutic efficacy. However, both models have intrinsic limitations, whilst the use of precision-cut tissue slices can bridge the gap between these mainstream models. Precision-cut tissue slices combine the advantage of high reproducibility, studying all cell sub-types whilst preserving the tissue matrix and extracellular architecture, thereby closely mimicking a mini-organ. This approach can be used to replicate the biological phenotype of liver monogenic diseases using mouse models., Methods: Here, we describe an optimised and easy-to-implement protocol for the culture of sections from mouse livers, enabling its use as a reliable ex-vivo model to assess the therapeutic screening of inherited metabolic diseases., Results: We show that precision-cut liver sections can be a reliable model for recapitulating the biological phenotype of inherited metabolic diseases, exemplified by common urea cycle defects such as citrullinemia type 1 and argininosuccinic aciduria, caused by argininosuccinic synthase (ASS1) and argininosuccinic lyase (ASL) deficiencies respectively., Conclusions: Therapeutic response to gene therapy such as messenger RNA replacement delivered via lipid nanoparticles can be monitored, demonstrating that precision-cut liver sections can be used as a preclinical screening tool to assess therapeutic response and toxicity in monogenic liver diseases., Competing Interests: Competing interests: The mRNA lipid nanoparticles were provided by Moderna. JB is receiving research funding from Moderna Therapeutics, (Copyright: © 2024 Perocheau D et al.)