The application of the self-care deficit nursing theory in adolescents with cystic fibrosis: A randomized controlled study.

PURPOSE: The present study evaluates the effects of the application of Self-Care Deficit Nursing Theory (SCDNT) on the self-care knowledge and skills in adolescents with Cystic Fibrosis. DESIGN AND METHODS: This randomized controlled study included 30 adolescents who were equally divided into the intervention and control groups. The data were collected by using the Sociodemographic Data Form and the Self-Care Knowledge and Skills Evaluation Forms for Adolescents with Cystic Fibrosis. A total of seven home visits were conducted with the adolescents in the intervention group, during which individualized care plans were applied based on the Self-Care Deficit Nursing Theory. The self-care needs of the adolescents in the control group were identified based on data collected during two home visits conducted at the onset of the study and after 4.5 months. RESULTS: As a result of the application of nursing interventions based on the SCDNT, a statistically significant increase was noted in the self-care knowledge and skills of the adolescents in the intervention group (p < 0.05). Additionally, after the interventions based on SCDNT, the need of adolescents for nursing interventions decreased significantly over time in all adolescents in the intervention group (p < 0.05). CONCLUSIONS: The application of nursing interventions based on the SCDNT was effective in enhancing the self-care knowledge and skills of adolescents with Cystic Fibrosis. PRACTICE IMPLICATIONS: Nurses can benefit from Orem's Self-Care Deficit Nursing Theory in the design and application of the individualized care of adolescents with Cystic Fibrosis to improve their self-care practices.

Functional evaluation of the tachycardia patient-derived iPSC cardiomyocytes carrying a novel pathogenic SCN5A variant.

Tachycardia is characterized by high beating rates that can lead to life-threatening fibrillations. Mutations in several ion-channel genes were implicated with tachycardia; however, the complex genetic contributors and their modes of action are still unclear. Here, we investigated the influence of an SCN5A gene variant on tachycardia phenotype by deriving patient-specific iPSCs and cardiomyocytes (iPSC-CM). Two tachycardia patients were genetically analyzed and revealed to inherit a heterozygous p.F1465L variant in the SCN5A gene. Gene expression and immunocytochemical analysis in iPSC-CMs generated from patients did not show any significant changes in mRNA levels of SCN5A or gross NaV1.5 cellular mislocalization, compared to healthy-derived iPSC-CMs. Electrophysiological and contraction imaging analysis in patient iPSC-CMs revealed intermittent fibrillation-like states, occasional arrhythmic events, and sustained high-paced contractions that could be selectively reduced by flecainide treatment. The patch-clamp analysis demonstrated a negative shift in the voltage-dependent activation at the patient-derived iPSC-CMs compared to the healthy control line, suggestive of a gain-of-function activity associated with the SCN5A+/p.F1465L variant. Our patient-derived iPSC-CM model recapitulated the clinically relevant characteristics of tachycardia associated with a novel pathogenic SCN5A+/p.F1465L variant leading to altered Na+ channel kinetics as the likely mechanism underlying high excitability and tachycardia phenotype.

Bioengineering human skeletal muscle models: Recent advances, current challenges and future perspectives.

Engineering models of human skeletal muscle tissue provides unique translational opportunities to investigate and develop therapeutic strategies for acute muscle injuries, and to establish personalised and precision medicine platforms for in vitro studies of severe neuromuscular and musculoskeletal disorders. Several myogenic and non-myogenic cell types can be isolated, generated, amplified and combined with scaffolds and biomaterials to achieve this aim. Novel bio-fabrication strategies, which include exogenous stimuli to enhance tissue maturation, promise to achieve an ever-increasing degree of tissue functionalisation both in vivo and in vitro. Here we review recent advances, current challenges and future perspectives to build human skeletal muscle tissue "in a dish", focusing on the cellular constituents and on applications for in vitro disease modelling. We also briefly discuss the impact that emerging technologies such as 3D bioprinting, organ-on-chip and organoids might have to circumvent technical hurdles in future studies.

The Effect of Self-Efficacy, Social Support and Quality of Life on Readiness for Transition to Adult Care Among Adolescents with Cystic Fibrosis in Turkey.

PURPOSE: The aim of this study is to examine the effects of self-efficacy, social support and quality of life on readiness for transition to adult care in adolescents with cystic fibrosis. DESIGN AND METHODS: A descriptive and cross-sectional study design was used. Data were collected from 50 adolescent between 14 and 17 years old with cystic fibrosis, by using The Transition Readiness Assessment Questionnaire, Social Support Appraisals Scale for Children, Self-Efficacy Questionnaire for Children and health-related quality-of-life instrument, the KIDSCREEN-10. RESULTS: A positive correlation was found between the readiness levels of adolescents for transition to adult care and self-efficacy levels. In path analysis, self-efficacy was found to have a significant effect on the level of readiness for transition to adult care. There was not statistically significant relationship between the level of readiness for transition to adult care and health-related quality of life and perceived social support. Path analysis revealed that health-related quality of life and perceived social support had significant effects on the self-efficacy levels of adolescents. CONCLUSIONS: Self-efficacy were associated with readiness for the transition to adult care. Although perceived social support and quality of life were not related with transition readiness these variables had significant effects on perceived self-efficacy, which was determined as a factor affecting the readiness for the transition to adult care. PRACTICE IMPLICATIONS: In adolescents with cystic fibrosis, self-efficacy, social support and quality of life levels should be taken into account when planning preparation programs for transition to adult care.