ABSTRACT
Objectives: Dysregulated RNA alternative splicing is the hallmark of myotonic dystrophy type 1 (DM1). However, the association between RNA mis-splicing and physical function in children with the most severe form of disease, congenital myotonic dystrophy (CDM), is unknown. Methods: 82 participants (42 DM1 adults & 40 CDM children) with muscle biopsies and measures of myotonia, motor function, and strength were combined from five observational studies. Data were normalized and correlated with an aggregate measure of alternative splicing dysregulation, [MBNL] inferred in skeletal muscle biopsies. Multiple linear regression analysis was performed to predict [MBNL] inferred using clinical outcome measures alone. Similar analyses were performed to predict 12-month physical function using baseline metrics. Results: Myotonia (measured via vHOT) was significantly correlated with RNA mis-splicing in our cross-sectional population of all DM1 individuals; CDM participants alone displayed no myotonia despite a similar range of RNA mis-splicing. Measures of motor performance and muscle strength were significantly associated with [MBNL] inferred in our cohort of all DM1 individuals and when assessing CDM children independently. Multiple linear regression analyses yielded two models capable of predicting [MBNL] inferred from select clinical outcome assessments alone in all subjects (adjusted R 2 = 0.6723) or exclusively in CDM children (adjusted R 2 = 0.5875). Interpretation: Our findings establish significant correlations between skeletal muscle performance and a composite measure of alternative splicing dysregulation, [MBNL] inferred, in DM1. The strength of these correlations and the development of the predictive models will assist in designing efficacious clinical trials for individuals with DM1, particularly CDM.
ABSTRACT
OBJECTIVE: The aim of this study was to examine the effect of an educational intervention on nurses' knowledge defined as understanding, beliefs, and actions to be taken, regarding nurse suicide. BACKGROUND: Between 2007 and 2018, nurses were 18% more likely to die by suicide than the general public. As a result, an assessment of nurses' knowledge regarding suicide has become an important issue for nursing administration. METHODS: A quasi-experiment (N = 225) was conducted. Variables of interest were assessed pre and post an educational intervention on 3 domains related to suicide. RESULTS: Significant and meaningful differences were found regarding 2 domains of interest in nurses' understanding related to nurse suicide, and actions to be taken, pre and post intervention. A significant change occurred regarding beliefs; however, the difference was small and therefore not meaningful. CONCLUSIONS: Finding demonstrate that educational interventions focused on issues related to suicide can enhance nurses' knowledge of the challenges implicit when one considers taking one's own life.