Hawaii J Health Soc Welf
December 2024
Commensality, the act of eating together, when organized around facilitated discussion is an evidence-based intervention that can promote engagement and reduce physician burnout. The purpose of this pilot study is to evaluate the feasibility, acceptance, and impact of a commensality intervention for physicians. The Commensality Intervention was based on a Mayo Clinic model that consisted of 6, 2-hour dinner meetings at local restaurants over 6 months with facilitated discussion.
View Article and Find Full Text PDFBackground: The Community Oriented Primary Care Project Model (COPC) has been well studied globally as well as in the United States as a way to provide both community-centered primary care and to engage in community based research. Rural trainees through the University of Illinois College of Medicine's Rural Medical Education Program (RMED) and Rural Pharmacy Education Program (RPHARM) students through the University of Illinois Chicago College of Pharmacy have participated in a COPC project as a capstone to a four month longitudinal, immersive community-based experience in a rural primary care setting. The purpose of this study was to assess the type of projects implemented through a rural health professions curriculum over a 30 year project period.
View Article and Find Full Text PDFAm J Respir Cell Mol Biol
October 2024
The lung is a vital organ that undergoes extensive morphological and functional changes during postnatal development. To disambiguate how different cell populations contribute to organ development, we performed proteomic and transcriptomic analyses of four sorted cell populations from the lung of human subjects aged 0 to 8 years-old with a focus on early life. The cell populations analyzed included epithelial, endothelial, mesenchymal, and immune cells.
View Article and Find Full Text PDFBackground: CDKL5 deficiency disorder (CDD) is an early-onset developmental and epileptic encephalopathy. While a subset of individuals is believed to experience comorbid behavioral disorders, none have reported well-defined affective disorders. Though there is a documented association between epilepsy and mood disorders, they may go undetected in the CDD population due to difficulty assessing mood in the presence of severe/profound intellectual disability and disease-related sleep dysregulation.
View Article and Find Full Text PDFAll talkers show some flexibility in their speech, and the ability to imitate an unfamiliar accent is a skill that shows vast individual differences. Yet the source of these individual differences, in particular whether they originate from perceptual, motor, or social/personality factors, is not yet clear. In the current study, we ask how individual differences in these factors predict individual differences in deliberate accent imitation.
View Article and Find Full Text PDFPathogenic variants in the cyclin-dependent kinase-like 5 (CDKL5) gene are associated with CDKL5 deficiency disorder (CDD), a severe X-linked developmental and epileptic encephalopathy. Deletions affecting the 5' untranslated region (UTR) of CDKL5, which involve the noncoding exon 1 and/or alternatively spliced first exons (exons 1a-e), are uncommonly reported. We describe genetic and phenotypic characteristics for 15 individuals with CDKL5 partial gene deletions affecting the 5' UTR.
View Article and Find Full Text PDFObjective: The CDKL5 Clinical Severity Assessment (CCSA) is a comprehensive, content-validated measurement tool capturing the diverse challenges of cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD), a genetically caused developmental epileptic encephalopathy (DEE). The CCSA is divided into clinician-reported (CCSA-Clinician) and caregiver-reported (CCSA-Caregiver) assessments. The aim of this study was to evaluate the factor structure of these measures through confirmatory factor analysis (CFA) and evaluate their validity and reliability.
View Article and Find Full Text PDFBackground: The maldistribution of pharmacy services in underserved areas is a national issue. Analysis of data from the 2019 National Pharmacist Workforce Study indicated that 13.9% of pharmacists were working in a rural community.
View Article and Find Full Text PDFBackground And Objective: CDKL5 deficiency disorder presents as a challenging condition with early-onset refractory seizures, severe developmental delays, and a range of other neurological symptoms. Our study aimed to explore the benefits and side effects of anti-seizure medications (ASMs) in managing seizures among individuals with CDKL5 deficiency disorder, drawing on data from the International CDKL5 Disorder Database.
Methods: Data for this retrospective cohort study were obtained from the International CDKL5 Disorder Database, which contains responses from a baseline questionnaire administered between 2012 and 2022 and a follow-up questionnaire administered between 2018 and 2019.
Study Objectives: Sleep difficulties are common in CDKL5 deficiency disorder, a developmental and epileptic encephalopathy. This study evaluated the factor structure of the Disorders of Initiating and Maintaining Sleep (DIMS), Disorders of Excessive Somnolence (DOES), and Sleep Breathing Disorders domains of the Sleep Disturbance Scale for Children for CDKL5 deficiency disorder.
Methods: A cross-sectional psychometric study design was used.
CDKL5 deficiency disorder (CDD) is a rare developmental and epileptic encephalopathy. Ganaxolone, a neuroactive steroid, reduces the frequency of major motor seizures in children with CDD. This analysis explored the effect of ganaxolone on non-seizure outcomes.
View Article and Find Full Text PDFBackground: Fetal alcohol spectrum disorders (FASD) are associated with neurodevelopmental challenges leading to difficulties with everyday life tasks. The Families Moving Forward (FMF) Program teaches caregivers to use positive behavior support (PBS), integrated with other techniques. However, it is unknown how caregivers retain and use these PBS strategies after the intervention.
View Article and Find Full Text PDFAm J Health Syst Pharm
October 2024
Purpose: The tele-intensive care unit (tele-ICU) pharmacist facilitates patient-specific diabetic ketoacidosis (DKA) treatment utilizing guideline-directed therapy. This study was designed to determine how patient-specific interventions by a tele-ICU pharmacist affected patients with DKA compared to the standard of care.
Methods: This retrospective cohort study utilized custom reports and manual chart review to evaluate the electronic health records of patients 18 years or older who received continuous intravenous insulin and were admitted for DKA between January 2019 and December 2020.
Interprofessional rounding is a common, yet unrequired, part of the pharmacy experiential curriculum. Little is known about the optimal interprofessional rounding structure for student pharmacists. A scoping review was performed to assess the amount and type of information available regarding student pharmacist participation on interprofessional rounding teams.
View Article and Find Full Text PDFObjectives: School-based mindfulness interventions in children have shown benefits to child well-being. Here, we investigated the effectiveness of a remote, app-based mindfulness intervention for promoting well-being in children.
Methods: We conducted a randomized controlled trial (RCT) with two control groups to examine the effects of an 8-week mindfulness intervention in U.
Alcohol Clin Exp Res (Hoboken)
April 2024
Viruses impact microbial systems through killing hosts, horizontal gene transfer, and altering cellular metabolism, consequently impacting nutrient cycles. A virus-infected cell, a "virocell," is distinct from its uninfected sister cell as the virus commandeers cellular machinery to produce viruses rather than replicate cells. Problematically, virocell responses to the nutrient-limited conditions that abound in nature are poorly understood.
View Article and Find Full Text PDFAm J Intellect Dev Disabil
March 2024
Loss of function variants in the Cyclin-dependent kinase-like 5 gene (CDKL5) causes CDKL5 deficiency disorder (CDD). Most cases of CDD are due to a de novo missense or truncating variants. The CDKL5 gene was discovered in 1998 as part of the genomic mapping of the chromosome Xp22 region that led to the discovery of the serine-threonine kinases STK9.
View Article and Find Full Text PDFObjective: Lead time to treatment (clinical onset of epileptic spasms [ES] to initiation of appropriate treatment) is known to predict outcomes in infantile epileptic spasms syndrome (IESS). Timing the clinical onset of ES is crucial to establish lead time. We investigated how often ES onset could be established to the nearest week.
View Article and Find Full Text PDFPurpose: Validated measures capable of demonstrating meaningful interventional change in the CDKL5 deficiency disorder (CDD) are lacking. The study objective was to modify the Rett Syndrome Gross Motor Scale (RSGMS) and evaluate its psychometric properties for individuals with CDD.
Methods: Item and scoring categories of the RSGMS were modified.
Multicellular rosettes are transient epithelial structures that serve as important cellular intermediates in the formation of diverse organs. Using the zebrafish posterior lateral line primordium (pLLP) as a model system, we investigated the role of the RhoA GEF Mcf2lb in rosette morphogenesis. The pLLP is a group of ∼150 cells that migrates along the zebrafish trunk and is organized into epithelial rosettes; these are deposited along the trunk and will differentiate into sensory organs called neuromasts (NMs).
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