2024 Update of the TSOC Expert Consensus of Fabry Disease.

As an X-linked inherited lysosomal storage disease that is caused by α-galactosidase A gene variants resulting in progressive accumulation of pathogenic glycosphingolipid (Gb3) accumulation in multiple tissues and organs, Fabry disease (FD) can be classified into classic or late-onset phenotypes. In classic phenotype patients, α-galactosidase A activity is absent or severely reduced, resulting in a more progressive disease course with multi-systemic involvement. Conversely, late-onset phenotype, often with missense variants (e.g., IVS4+919G>A) in Taiwan, may present with a more chronic clinical course with predominant cardiac involvement (cardiac subtype), as they tend to have residual enzyme activity, remaining asymptomatic or clinically silent during childhood and adolescence. In either form, cardiac hypertrophy remains the most common feature of cardiac involvement, potentially leading to myocardial fibrosis, arrhythmias, and heart failure. Diagnosis is established through α-galactosidase enzyme activity assessment or biomarker analyisis (globotriaosylsphingosine, Lyso-Gb3), advanced imaging modalities (echocardiography and cardiac magnetic resonance imaging), and genotyping to differentiate FD from other cardiomyopathy. Successful therapeutic response relies on early recognition and by disease awareness from typical features in classic phenotype and cardiac red flags in cardiac variants for timely therapeutic interventions. Recent advances in pharmacological approach including enzyme replacement therapy (agalsidase alfa or beta), oral chaperone therapy (migalastat), and substrate reduction therapy (venglustat) aim to prevent from irreversible organ damage. Genotype- and gender-based monitoring of treatment effects through biomarker (Lyso-Gb3), renal assessment, and cardiac responses using advanced imaging modalities are key steps to optimizing patient care in FD.

Social network associated with depressed mood and sarcopenia among older adults in Taiwan.

BACKGROUND: Social isolation is increasing in aging societies; however, its relationship with depressed mood and sarcopenia is not well studied. This study aims to examine the influence of social network on depressed mood and sarcopenia among community-dwelling older adults in Taiwan. METHODS: We collected data from a sample of 981 older adults residing in the community. These individuals received government-subsidized preventive healthcare services for adults at a district hospital in Taipei in 2021. The social network of the older adults who participated was assessed using the Lubben Social Network Scale, while depressed mood was assessed using the Geriatric Depression Scale. The definition of sarcopenia used in this study was based on the 2019 Asian Working Group for Sarcopenia. RESULTS: According to this study, sarcopenia was present in approximately 15 % of older adults. Multiple logistic regression analysis showed that older adults who had poor social network and did not meet the recommended 150 min of regular physical activity per week were more likely to have depressed mood. Additionally, older adults who were older, underweight, did not engage in regular physical activity, and had poor social network were more likely to have sarcopenia. CONCLUSION: Poor social network was associated with increased risks of depressed mood and sarcopenia among older adults.

Relationship between physical activity and perceptions of ageing from the perspective of healthy ageing among older people with frailty with chronic disease: a cross-sectional study.

BACKGROUND: In Taiwan, the 2019 Elderly Frailty Assessment found that 11.2% of older people have frailty problems. Some researchers have found that older persons' negative perspectives on ageing aggravate the progression of frailty, thereby increasing their risk of disability. This study aimed to investigate associations of physical activity and perceptions of ageing on perspectives of healthy ageing in older people with frailty and chronic diseases and to compare the differences in their frailty status. METHODS: This study used a descriptive cross-sectional design. Participants were recruited from community long-term care stations. The inclusion criteria were (1) no severe cognitive impairment and ability to communicate in Mandarin and Taiwanese; (2) over 65 years old; (3) at least one chronic disease; and (4) at least one debilitating item in the Study of Osteoporotic Fracture index. A total of 312 participants were recruited. The Brief Ageing Perceptions Questionnaire Chinese version, Healthy Ageing Perspectives Questionnaire, and Physical Activity Scale for the Elderly Chinese Version were used for measurement. RESULTS: The study results found that demographic variables, perceptions of ageing, and physical activity were significantly correlated with perspectives on healthy ageing, including age, Activities of Daily Living, education, all domains of perceptions of ageing, and household- and work-related physical activity. With regard to the frailty status level, prefrailty was better than frailty from the perspective of healthy ageing in older people with chronic disease (t = 5.35, p < 0.05). Hierarchical regression analysis was used to predict the healthy ageing perspectives of older persons with chronic disease involving a chronic time-line, positive control, health-related changes, and work-related activities. Those domains could predict 21% of the variance in healthy ageing perspectives. CONCLUSION: It is suggested that in community long-term care stations, health care providers can arrange activities to improve the perception of ageing that are acceptable for older people with frailty and chronic diseases and encourage older people to participate in service activities to achieve a sense of social participation.

[The Legal Risks Faced in Nursing and Smart Healthcare].

Smart healthcare and telemedicine are shortening the gap in the quality of medical and healthcare available to urban and rural communities and allowing care to be provided free of space and time limitations. The COVID-19 pandemic has hastened the general adoption of telemedicine as a protocol in hospitals and healthcare and led to a rapid paradigm shift from medical expertise to telemedicine eHealth. Because nurses play a significant role as members of interdisciplinary teams, we are expected to quickly adapt to and use artificial intelligence and other new technology innovations. This article elucidates the intellectual-property-right-related responsibilities and obligations of nurses and attendant legal risks in the context of smart medical research, clinical care standards, information laws and rules, and related policymaking. In addition, the legal issues related to telemedicine are discussed and analyzed.

Diagnostic Potential of Combined Photoplethysmography and Ankle-Brachial Index in Peripheral Arterial Disease: A Duplex Ultrasonography-Based Comparative Study.

BACKGROUND: Although ankle-brachial index (ABI) and photoplethysmography (PPG) have also shown adequate sensitivity in detecting peripheral arterial disease, their diagnostic performance is less reliable in asymptomatic cases or those with high atherosclerotic cardiovascular risks. METHODS AND RESULTS: We evaluated 130 participants using ABI, PPG, and duplex ultrasonography, diagnosing 65 with peripheral arterial disease. From the PPG, we derived 2 parameters: PPG amplitude ratio of the lower-to-upper extremities (PPGratio) and the PPG amplitude of the lower extremity (PPGamp). Sensitivity, specificity, accuracy, and the area under receiver operating characteristic (ROC) curve were calculated for PPG parameters and ABI, and their combination of both methods. Univariate and multivariate logistic regression assessed the prognostic potential of these parameters. ROC analysis revealed optimal cutoff values in diagnosing peripheral arterial disease were 0.417 for PPGratio and "58" for PPGamp. Both PPGratio and PPGamp demonstrated significantly higher sensitivities, 78.4% and 75.7%, respectively, compared with 55.9% for ABI <0.9 (P<0.05). The areas under the ROC curves of combination models, including model 1 (ABI <0.9 and PPGratio), model 2 (ABI <0.9 and PPGamp), and model 3 (ABI <0.9, PPGratio, and PPGamp), exhibited improved performance with areas under the ROC curves of 0.922, 0.922, and 0.931 (all P<0.01) compared with ABI alone (area under the ROC curve, 0.822). Additionally, the PPG parameters, both alone and combined with ABI, were associated with major adverse cardiac events and all-cause mortality after adjusting for other relevant factors. CONCLUSIONS: On the basis of duplex ultrasonography, combining ABI and PPG markedly improves peripheral arterial disease diagnosis in high-risk individuals compared with either method alone and provides crucial insights into major adverse cardiac events and all-cause mortality risks.

Advancing Transportation Equity and Safety Through Autonomous Vehicles.

Motor vehicle crashes are a leading cause of death in the United States, and disproportionately impact communities of color. Replacing human control with automated vehicles (AVs) holds the potential to reduce crashes and save lives. The benefits of AVs, including automated shuttles, buses, or cars could extend beyond safety to include improvements in congestion, reductions in emissions, and increased access to mobility, particularly for vulnerable populations. However, AVs have not attained the level of public trust that has been expected, given their potential to save lives and increase access to mobility. Public opinion surveys have highlighted safety and security concerns as reasons for this lack of confidence. In this study, we present the findings of an experiment we conducted to actively shift mindsets on AVs toward advancing health equity. We demonstrate through a nationally representative sample of 2265 U.S. adults that the public support for AVs can be improved by expanding their scope of application to include advancing social benefit. The survey began with questions on respondent's support for AVs based on a priori knowledge and beliefs. Consistent with prior surveys, baseline support (strong support and some degree of support) was low at 26.4% (95% confidence interval 24.0-29.0). After introducing information about how AVs could be used to provide mobility for older adults, those with limited income, or the vision-impaired, respondents were asked to reassess their support for AVs. Support significantly increased to include the majority of respondents. By prioritizing the deployment of AVs to serve individuals and communities in greatest need of mobility, AVs would not only demonstrate compelling social value by reducing disparities but would also gain widespread public support among the U.S. public.

Speeding behavior among teenage drivers during the learner and early independent driving stage: A case study approach.

INTRODUCTION: Speed is a primary contributing factor in teenage driver crashes. Yet, there are significant methodological challenges in measuring real-world speeding behavior. METHOD: This case study approach analyzed naturalistic driving data for six teenage drivers in a longitudinal study that spanned the learner and early independent driving stages of licensure in Maryland, United States. Trip duration, travel speed and length were recorded using global position system (GPS) data. These were merged with maps of the Maryland road system, which included posted speed limit (PSL) to determine speeding events in each recorded trip. Speeding was defined as driving at the speed of 10 mph higher than the posted speed limit and lasting longer than 6 s. Using these data, two different speeding measures were developed: (1) Trips with Speeding Episodes, and (2) Verified Speeding Time. Conclusions & Practical Applications: Across both measures, speeding behavior during independent licensure was greater than during the learner period. These measures improved on previous methodologies by using PSL information and eliminating the need for mapping software. This approach can be scaled for use in larger samples and has the potential to advance understanding about the trajectory of speeding behaviors among novice teenage drivers.

Development of an algorithm for analysis of routes: Case studies using novice and older drivers.

INTRODUCTION: This study addresses the lack of methods to quantify driver familiarity with roadways, which poses a higher risk of crashes. METHOD: We present a new approach to assessing driving route diversity and familiarity using data from the DrivingApp, a smartphone-based research tool that collects trip-level information, including driving exposure and global positioning system (GPS) data, from young novice drivers (15-19 years old) to older drivers (67-78 years old). Using these data, we developed a GPS data-based algorithm to analyze the uniqueness of driving routes. The algorithm creates same route trip (SRT) arrays by comparing each trip of an identified user, employing statistically determined thresholds for GPS coordinate proximity and trip overlap. The optimal thresholds were established using a General Linear Model (GLM) to examine distance, and repeated observations. The Adjusted Breadth-First Search method is applied to the SRT arrays to prevent double counting or trip omission. The resulting list is classified as geographically distinct routes, or unique routes (URs). RESULTS: Manual comparison of algorithm output with geographical maps yielded an overall precision of 0.93 and accuracy of 0.91. The algorithm produces two main outputs: a measure of driving diversity (number of URs) and a measure of route-based familiarity derived from the Rescorla-Wagner model. To evaluate the utility of these measures, a Gaussian mixture model clustering algorithm was used on the young novice driver dataset, revealing two distinct groups: the low-frequency driving group with lower route familiarity when having higher route diversity, whereas the high-frequency driving group with the opposite pattern. In the older driver group, there was a significant correlation found between the number of URs and Geriatric Depression Score, or walking gait speed. PRACTICAL APPLICATIONS: These findings suggest that route diversity and familiarity could complement existing measures to understand driving safety and how driving behavior is related to physical and psychological outcomes.

The effectiveness of visual art on environment in nursing home.

PURPOSE: This Taiwan study investigated the effect of a visual art-based friendly environment on nursing home residents' satisfaction with their living environment. DESIGN: A pre-experimental design was used. Thirty-three residents in a nursing home were recruited in a one-group pre- and post-test study. METHODS: The four-floor living environment was integrated using visual art, reminiscence, and gardening based on the local culture and history. Each floor was given a different theme, one that was familiar to most of the residents on the floor. The Satisfaction with Living Environment at Nursing Home Scale (SLE-NHS) was developed to measure outcomes. FINDINGS: Of the 33 participants recruited, 27 (81.8%) were women and 6 (18.2%) were men. Their mean age was 79.24 ± 7.40 years, and 48.5% were severely dependent in activities of daily living. The SLE-NHS showed adequate reliability and validity. Its three domains were generated and defined using factor analysis. After the visual art-based intervention, the score on the "recalling old memories" subscale was significantly higher (t = -13.32, p < .001). However, there were no significant score changes on the "convenience" and "pretty and pleasurable" subscales. In general, the participants were satisfied with the redesigned environment and felt happy in the sunny rooms. CONCLUSIONS: Visual art in a nursing home is a novel method for representing the local culture and stressing the spiritual value of the elderly residents who helped create it. Older adults' aesthetic activities through visual art, including reminiscence and local culture, may enrich their spirits in later life. CLINICAL RELEVANCE: Older adults' aesthetic activities through visual art have been shown to improve their satisfaction with their living environment. The SLE-NHS is a useful tool for evaluating their satisfaction.

Percutaneous Balloon Catheter Occlusion to Treat Extravasation from an Inferior Vena Cava Laceration Associated With a Pelvic Fracture Following Blunt Abdominal Trauma: A Case Report.

Blunt abdominal injury with pelvic fracture is common in polytrauma cases and is a major challenge for emergency physicians. Fluid resuscitation and massive transfusion protocol should be activated when pelvic fracture patients are found in hypovolemic shock. At the emergency department, resuscitative endovascular balloon occlusion of the aorta may be performed to temporarily control bleeding. Finally, a damage control operation or trans-arterial embolization may be performed in the hybrid operating room.

Susceptibility to hypertension based on MTHFR rs1801133 single nucleotide polymorphism and MTHFR promoter methylation.

Background: The aetio-pathologenesis of hypertension is multifactorial, encompassing genetic, epigenetic, and environmental factors. The combined effect of genetic and epigenetic changes on hypertension is not known. We evaluated the independent and interactive association of MTHFR rs1801133 single nucleotide polymorphism (SNP) and MTHFR promoter methylation with hypertension among Taiwanese adults. Methods: We retrieved data including, MTHFR promoter methylation, MTHFR rs1801133 genotypes (CC, CT, and TT), basic demography, personal lifestyle habits, and disease history of 1,238 individuals from the Taiwan Biobank (TWB). Results: The distributions of hypertension and MTHFR promoter methylation quartiles (ß < 0.1338, 0.1338 ≤ ß < 0.1385, 0.1385 ≤ ß < 0.1423, and ß ≥ 0.1423 corresponding to

[A "dialogue" between the aesthetics of nursing and philosophy].

Nursing aesthetics belong to the broader school of aesthetics, a branch of philosophy, as well as the nursing arts, an element of professional nursing. The philosophy of aesthetics recognizes the connection between an author and appreciators and identifies both substantive and abstract aesthetic experiences in interpersonal communication through the fine arts. Nursing aesthetics values the meaningful moments of patients, is sensitive to the influences of different circumstances and situations, and appreciates the unique qualities of humanness. Nursing aesthetics is emancipatory knowledge and involves empirical, ethical and personal knowing. The article is based on a search of OvidSP and Chinese Electronic Periodical Services (CEPS) database references using key words including aesthetic, aesthetics, art of nursing, or nursing aesthetics as well as a review of books related to aesthetics, knowledge construction, and nursing aesthetics. Authors determined definitions as defined by nursing experts and the applications thereof in clinical practice. This article aimed to illustrate that the ultimate concern of philosophy is "goodness" and that the foundation of caring behaviors is "love". In practice, nursing aesthetics is expressed through empathy, appreciation, inspiration and the therapeutic use of the self. Through aesthetic knowing and enhanced perceptual sensibility and reflection, nurses can transform intuitive knowing into art-acts and ultimately enhance nursing care quality.

Agent-Based Modeling for Implementation Research: An Application to Tobacco Smoking Cessation for Persons with Serious Mental Illness.

BACKGROUND: Implementation researchers have sought ways to use simulations to support the core components of implementation, which typically include assessing the need for change, designing implementation strategies, executing the strategies, and evaluating outcomes. The goal of this paper is to explain how agent-based modeling could fulfill this role. METHODS: We describe agent-based modeling with respect to other simulation methods that have been used in implementation science, using non-technical language that is broadly accessible. We then provide a stepwise procedure for developing agent-based models of implementation processes. We use, as a case study to illustrate the procedure, the implementation of evidence-based smoking cessation practices for persons with serious mental illness (SMI) in community mental health clinics. RESULTS: For our case study, we present descriptions of the motivating research questions, specific models used to answer these questions, and a summary of the insights that can be obtained from the models. In the first example, we use a simple form of agent-based modeling to simulate the observed smoking behaviors of persons with SMI in a recently completed trial (IDEAL, Comprehensive Cardiovascular Risk Reduction Trial in Persons with SMI). In the second example, we illustrate how a more complex agent-based approach that includes interactions between patients, providers and site administrators can be used to provide guidance for an implementation intervention that includes training and organizational strategies. This example is based in part on an ongoing project focused on scaling up evidence-based tobacco smoking cessation practices in community mental health clinics in Maryland. CONCLUSION: In this paper we explain how agent-based models can be used to address implementation science research questions and provide a procedure for setting up simulation models. Through our examples, we show how what-if scenarios can be examined in the implementation process, which are particularly useful in implementation frameworks with adaptive components.

Chest pain after percutaneous coronary intervention in patients with stable angina.

BACKGROUND: Percutaneous coronary intervention (PCI) has been widely used to treat acute coronary syndrome but is only recommended as an additional treatment to medical therapy and risk modification in patients with refractory or progressing angina. The number of PCI in this patient population is still increasing. Post-PCI chest pain (PPCP) is one of the common problems of PCI. Its presentation and causes in patients with stable angina are poorly understood. PATIENTS AND METHODS: This study retrospectively collected clinical information of 167 patients who had stable angina and underwent elective PCI, including 70 patients with PPCP 24 hours after procedure and 97 patients without PPCP. The incidence and predictors of PPCP were analyzed. RESULTS: The incidence of PPCP was 41.9% (70/167). Compared with non-PPCP patients, PPCP patients had more abnormal post-PCI electrocardiogram (ECG) changes (new Q-waves, ST-segment shifts, or T-waves inversion) and serum cardiac troponin I (cTnI) elevation, more PCI vessels, and stent placement (all P<0.05). More PPCP patients required repeat revascularization than non-PPCP patients after PCI (P=0.043). PPCP was correlated with abnormal post-PCI ECG changes (P<0.0001), cTnI elevation (P<0.0001), post-PCI serum level of cTnI (P<0.0001), number of stents placed (P=0.009), and pre-PCI cTnI level (P=0.049). The strongest predictors of PPCP were abnormal post-PCI ECG changes (P<0.0001), post-PCI cTnI level (P<0.0001), and cTnI elevation (P<0.0001), followed by the number of stents placed (P=0.048). CONCLUSION: PPCP is common in patients with stable angina in our cohort. It is associated with abnormal ECG changes, cTnI elevation, and number of stents placed.

Pediatric otitis media in Fiji: Survey findings 2015.

OBJECTIVE: Otitis media (OM), as a common infectious disease, is a major cause of hearing impairment among the general population. OM remains a major public health threat in the Pacific islands, but the risks of OM have not been thoroughly explored in this region. The objective of this study is to investigate the prevalence, clinical features, and quality-of-life impacts of OM in Fiji. METHODS: In the medical service trip entitled "Healing and Hope - Taiwan Cathay Heart and Hearing Medical Mission to Fiji" (TCHHMMF), we conducted a cross-sectional OM survey study in Suva and Sigatoka areas (Korolevu, Cuvu, and Lomawai) in the summer of 2015. The otitis media - 6 (OM-6) was used to survey the OM-related quality of life. RESULTS: In the 467 pediatric patients (aged 0-18 years old) screened, 13 (2.78%) have acute otitis media (AOM), 37 (7.92%) have otitis media with effusion (OME), and 19 (4.1%) have chronic otitis media (COM). Age (OR 0.53, 95% CI: 0.36-0.77) is a significant predictor of AOM, whereas male gender (OR 2.46, 95% CI: 1.13-5.37), smoke exposure (OR 2.81, 95% CI: 1.01-7.82), and concomitant chronic sinusitis (OR 6.05, 95% CI: 2.31-15.88) are significant predictors of OME. The mean OM-6 item scores are highest in caregiver concerns (3.8), physical suffering (3.7), and hearing loss (3.4) domains. CONCLUSION: OM is an important primary care disease in Fiji that remains under-served. It is critical to educate professionals, parents, and patients to detect and to improve care for OM.

Managing problem behaviors in cognitively impaired older people: the competence and preparedness of healthcare providers in long-term care facilities.

BACKGROUND: The prevelance of cognitive impairment in older individuals is increasing in Taiwan. Most healthcare providers (HCPs) experience deep frustration while caring for residents of long-term care facilities who are cognitively impaired. Competence and preparedness of HCPs represent key factors in being able to successfully create and maintain a safe environment for elderly wards and are one of the main concerns of cognitive impairment care. PURPOSE: The purpose of this study was to investigate the competence and preparedness of HCPs to manage the problem behaviors of cognitively impaired elders living in long-term care facilities. METHODS: This study utilized a correlational research design and included 221 participants that were recruited using a stratified random sampling process. Structured and semistructured questionaires on competence and preparedness of caring for cognitively impaired elders were used to collect data. Descriptive statistics and univariate and multivariate analyses were used to analyze the relationship between HCP demographic characteristics and their competence or preparedness in managing cognitive impairment care. RESULTS: Ninety-five percent of participants were women. The top scoring item in the competence dimension was "preventing problem behaviors from occurring" (M +/- SD = 3.89 +/- 2.89), and the lowest scoring item was "reading related books and articles" (2.91 +/- 0.85). In the preparedness dimension, "modification of environment" (3.22 +/- 1.07) was the highest scoring item, and "resources referral skills" was the lowest scoring item (3.02 +/- 1.03). Results showed supervisors (F = 7.89, p = .001) and college graduates (F = 2.89, p = .047) to have higher levels of competency than other participants. Participant knowledge level in dealing with problem behaviors was significantly and positively correlated with frequency of continuing education participation (F = 28.73, p < .001). CONCLUSIONS/IMPLICATIONS FOR PRACTICE: Results point to the need for continuing education, practicum, counseling, staffing resources, and involvement of other professionals to increase HCP readiness and competence.