The effects of singing interventions on quality of life, mood and levels of agitation in community-dwelling people living with dementia: A quantitative systematic review.

Background and Aims: Music-based interventions have been found to benefit people living with dementia and have positive impacts on cognition and well-being. Most people with dementia live in the community and compared to people with dementia in residential care often have less access to music-based interventions. There are many forms of music interventions and singing has shown particular promise; in the realm of music interventions. It is important to determine what aspects of music interventions yield the most benefits for people with dementia. This review aimed to synthesise evidence on the impacts of singing interventions on quality of life, mood and neuropsychiatric symptoms for community-dwelling people with dementia. Methods: We systematically searched three electronic databases (PsycINFO, MEDLINE and Web of Science) for studies reporting on singing interventions with community-dwelling people with dementia. Studies were eligible for inclusion if they reported on a singing intervention with people living with dementia that included an outcome measure of quality of life, mood or agitation. Fourteen publications were identified and included in this review, with a total of n = 361 people with dementia. Results: Despite some inconsistencies across the literature, evidence suggests that singing interventions led to an improvement in mood and a reduction in agitation levels in people living with dementia. There was no strong evidence to suggest that singing interventions led to significant improvements in quality of life. Conclusions: This review highlights the potential of singing interventions as an effective psychosocial intervention for community-dwelling people with dementia. For key developments in this area, we urge that future studies include a control group where possible which will allow for more robust examinations of singing interventions and allow intervention effects to be distinguished from general deterioration in dementia symptoms over time.

Regional trends in mental health inequalities in young people aged 16-25 in the UK and the role of cuts to local government expenditure: Repeated cross-sectional analysis using the British household panel Survey/UK household longitudinal survey.

Young people's mental health globally has been in decline. Because of their low perceived need, young people's services tend to be the first cut when budgets are reduced. There is a lack of evidence on how a reduction in services and opportunities for young people is associated with their mental health. Additionally, how this may be magnified by place and the assets and challenges of place. The aim of this study is to explore trends in young people's mental health measured by GHQ-12 over time in the twelve regions of the UK. We estimated an interrupted time series model using 2010 as a break point from which there was a shift in government policy to a prolonged period of large reductions in central government funding. Repeated cross-sectional data on young people aged 16-25 is used from the British Household Panel Survey and its successor survey UK Household Longitudinal Survey. Results showed a statistically significant reduction in mental health for young people living in the North East, Wales, and the East of England. The North East was the region with the largest reduction in funding and saw the greatest reduction in young people's mental health. Next, we look at how reductions in local government expenditure related to services for children and young people: children's social services, education, transportation, and culture; explain the observed decline in mental health. We employ a Blinder-Oaxaca Decomposition approach comparing young people's mental health between 2011 and 2017. Results show a marginally statistically significant decrease in young people's mental health over this time. Unobserved factors related to transport spending and children's social services explain some of this gap. Area level factors such as deprivation, infrastructure, and existing assets need to be considered when distributing funding for young people's services to avoid exacerbating regional inequalities in mental health.

Time series analysis of COVID-19's impact on physician and dentist visits in Iran.

This study aimed to assess the impact of the COVID-19 pandemic on general practitioner (GP), specialist, and dentist visits among 40 million Iranians covered by the Social Security Organization (SSO). A monthly interrupted time series analysis was conducted over a period of 72 months, including-47 months before the pandemic and 25 months after its onset. The outcomes variables were monthly number of GP, specialist, and dentist visits per 1000 SSO-insured individuals. The analysis was performed by total visits, visits to the SSO direct sector, and visits to the indirect sectors. The study found that in the first month of the pandemic, the number of visits per 1000 insured individuals significantly decreased for visits to GPs (by 51.12, 95% CI -64.42 to -37.88), visits to specialists (by 39.11, 95% CI -51.61 to -26.62), and visits to dentists (by 6.67, 95% CI -8.55 to -4.78). However, during the subsequent months of the pandemic, there was a significant increase in the number of monthly visits for all three categories, with GPs experiencing the highest increase (1.78 visits per 1000 insured), followed by specialists (1.32 visits per 1000 insured), and dentists (0.05 visits per 1000 insured). Furthermore, prior to the pandemic, the number of monthly GP visits per 1000 insured individuals was statistically significantly lower in the indirect sector compared to the direct sector (45.79, 95% CI -52.69 to -38.89). Conversely, the direct sector exhibited lower rates of specialist visits (25.84 visits per 1000 insured individuals, 95% CI 22.87 to 28.82) and dentist visits (0.75 visits per 1000 insured individuals, 95% CI 0.12 to 1.36) compared to the indirect sector. Additionally, the study found that in the first month of the pandemic, the monthly number of GP visits in the indirect sector significantly increased by 34.44 times (95% CI 24.81 to 44.08) compared to the direct sector. For specialist visits and dentist visits, the increase was 3.41 (95% CI -5.87 to 12.69) and 5.01 (95% CI 3.48 to 6.53) per 1000 insured individuals, respectively. Overall, the findings of this study demonstrate statistically significant disruptions in GP, specialist, and dentist visits during the COVID-19 pandemic, although some recovery was observed. Both the direct and indirect sectors experienced decreased visits.

Being able to be myself: Understanding autonomy and autonomy-support from the perspectives of autistic adults with intellectual disabilities.

LAY ABSTRACT: Autistic young adults with intellectual disabilities want to be autonomous but are less autonomous than other people. However, they can be autonomous with appropriate support. We wanted to learn how we can support autistic adults with intellectual disabilities to be more autonomous. We designed our study with help from five autistic community partners to make sure the research was relevant to autistic people and would improve their lives. We talked with eight autistic young adults with intellectual disabilities about autonomy. We defined "talk" as verbal language, as well as non-verbal cues such as body language, facial expressions, vocalizations, and laughter. We did art projects and played games while we talked. We met in small groups over multiple sessions. Our participants told us that being autonomous meant being able to be themselves. They told us three main ways to support their autonomy: (1) having choice and control, (2) being able to communicate in their own way, and (3) being in a safe environment. Families, support staff, and caregivers can use this information to help autistic young adults with intellectual disabilities to be autonomous.

Beyond the surface: a color-inclusive guide to central line site assessment.

Significant gaps exist in representation of diverse populations in central-line assessment education and tools. We review some of these gaps and provide some real-world guidance on how to assess central line sites in patients of all skin tones.

Obesity-Preventive Behaviors and Improvements in Depression among Diverse Utah Women Receiving Coaching from Community Health Workers.

OBJECTIVES: This study evaluated how high versus low-intensity community wellness coaching and health behaviors were associated with changes in depression screen results over one year. METHODS: This was an analysis of secondary data collected in a 12-month obesity-related community health worker (CHW) program for 485 Utah women of color. Depression screen (Patient Health Questionnaire-2 score ³3) and self-reported fruit/vegetable consumption and physical activity (FV/PA) were recorded quarterly. Associations between FV/PA and changes in depression screen over time were evaluated in multivariable models. RESULTS: Positive depression screen prevalence declined over 12 months (21.7% to 9.5%) with no difference between study arms. Overall, FV ³5 times/day (AOR=1.5; 95% CI 1.0-2.2), any PA (AOR=3.1; 95% CI 1.5-6.4), and muscle strengthening activities (AOR=1.13; 95% CI 1.01-1.26) were associated with improved depression screen results over time. CONCLUSION: These results indicate value in addressing and evaluating depression in obesity-related interventions in underserved communities.

Higher precision, first tier newborn screening for metachromatic leukodystrophy using 16:1-OH-sulfatide.

Newborn screening (NBS) for metachromatic leukodystrophy (MLD) is based on first-tier measurement of sulfatides in dried blood spots (DBS) followed by second-tier measurement of arylsulfatase A in the same DBS. This approach is very precise with 0-1 false positives per ∼30,000 newborns tested. Recent data reported here shows that the sulfatide molecular species with an α-hydroxyl, 16­carbon, mono-unsaturated fatty acyl group (16:1-OH-sulfatide) is superior to the original biomarker 16:0-sulfatide in reducing the number of first-tier false positives. This result is consistent across 4 MLD NBS centers. By measuring 16:1-OH-sulfatide alone or together with 16:0-sulfatide, the estimated false positive rate is 0.048% and is reduced essentially to zero with second-tier arylsulfatase A activity assay. The false negative rate is predicted to be extremely low based on the demonstration that 40 out of 40 newborn DBS from clinically-confirmed MLD patients are detected with these methods. The work shows that NBS for MLD is extremely precise and ready for deployment. Furthermore, it can be multiplexed with several other inborn errors of metabolism already tested in NBS centers worldwide.

Divergent role of Mitochondrial Amidoxime Reducing Component 1 (MARC1) in human and mouse.

Recent human genome-wide association studies have identified common missense variants in MARC1, p.Ala165Thr and p.Met187Lys, associated with lower hepatic fat, reduction in liver enzymes and protection from most causes of cirrhosis. Using an exome-wide association study we recapitulated earlier MARC1 p.Ala165Thr and p.Met187Lys findings in 540,000 individuals from five ancestry groups. We also discovered novel rare putative loss of function variants in MARC1 with a phenotype similar to MARC1 p.Ala165Thr/p.Met187Lys variants. In vitro studies of recombinant human MARC1 protein revealed Ala165Thr substitution causes protein instability and aberrant localization in hepatic cells, suggesting MARC1 inhibition or deletion may lead to hepatoprotection. Following this hypothesis, we generated Marc1 knockout mice and evaluated the effect of Marc1 deletion on liver phenotype. Unexpectedly, our study found that whole-body Marc1 deficiency in mouse is not protective against hepatic triglyceride accumulation, liver inflammation or fibrosis. In attempts to explain the lack of the observed phenotype, we discovered that Marc1 plays only a minor role in mouse liver while its paralogue Marc2 is the main Marc family enzyme in mice. Our findings highlight the major difference in MARC1 physiological function between human and mouse.

Improving newborn screening test performance for metachromatic leukodystrophy: Recommendation from a pre-pilot study that identified a late-infantile case for treatment.

Metachromatic leukodystrophy (MLD) is a devastating rare neurodegenerative disease. Typically, loss of motor and cognitive skills precedes early death. The disease is characterised by deficient lysosomal arylsulphatase A (ARSA) activity and an accumulation of undegraded sulphatide due to pathogenic variants in the ARSA gene. Atidarsagene autotemcel (arsa-cel), an ex vivo haematopoietic stem cell gene therapy was approved for use in the UK in 2021 to treat early-onset forms of pre- or early-symptomatic MLD. Optimal outcomes require early diagnosis, but in the absence of family history this is difficult to achieve without newborn screening (NBS). A pre-pilot MLD NBS study was conducted as a feasibility study in Manchester UK using a two-tiered screening test algorithm. Pre-established cutoff values (COV) for the first-tier C16:0 sulphatide (C16:0-S) and the second-tier ARSA tests were evaluated. Before the pre-pilot study, initial test validation using non­neonatal diagnostic bloodspots demonstrated ARSA pseudodeficiency status was associated with normal C16:0-S results for age (n = 43) and hence not expected to cause false positive results in this first-tier test. Instability of ARSA in bloodspot required transfer of NBS bloodspots from ambient temperature to -20°C storage within 7-8 days after heel prick, the earliest possible in this UK pre-pilot study. Eleven of 3687 de-identified NBS samples in the pre-pilot were positive for C16:0-S based on the pre-established COV of ≥170 nmol/l or ≥ 1.8 multiples of median (MoM). All 11 samples were subsequently tested negative determined by the ARSA COV of <20% mean of negative controls. However, two of 20 NBS samples from MLD patients would be missed by this C16:0-S COV. A further suspected false negative case that displayed 4% mean ARSA activity by single ARSA analysis for the initial test validation was confirmed by genotyping of this NBS bloodspot, a severe late infantile MLD phenotype was predicted. This led to urgent assessment of this child by authority approval and timely commencement of arsa-cel gene therapy at 11 months old. Secondary C16:0-S analysis of this NBS bloodspot was 150 nmol/l or 1.67 MoM. This was the lowest result reported thus far, a new COV of 1.65 MoM is recommended for future pilot studies. Furthermore, preliminary data of this study showed C16:1-OH sulphatide is more specific for MLD than C16:0-S. In conclusion, this pre-pilot study adds to the international evidence that recommends newborn screening for MLD, making it possible for patients to benefit fully from treatment through early diagnosis.

The Psychiatric Care of Children and Young Adults With Neurodegenerative Diseases.

Pediatric neurodegenerative disorders (PNDs), such as juvenile neuronal ceroid lipofuscinosis (CLN3 disease, also called Batten disease) and juvenile Huntington disease, are devastating conditions that result in progressive neurological dysfunction and profound medical comorbidities leading to early mortality in children and young adults.1 There are more than 70 PNDs, with a combined estimated prevalence of ∼0.1 in 1,000 live births.2,3 Individuals with PNDs commonly experience complex neuropsychiatric manifestations such as neurocognitive symptoms (dementia), irritability, aggression and self-injury, mood disorders, sensory alterations, and psychosis. Symptoms are dynamic, changing with illness progression, and evolve over time. Effects on patients and families can be devastating, and caregiver burden is enormous.4 We are a group of colleagues with backgrounds in pediatric neuropsychiatry, pediatric neuropalliative care, and pediatric neurology who care for patients together in specialized clinics.

Injury patterns of patients presenting to a non-governmental hospital in Western Uganda

Introduction: Injury is a leading cause of morbidity and mortality globally and disproportionately affects low-income countries. While most injury data comes from tertiary care centers in urban settings, the purpose of this study was to describe the characteristics and severity of injury in rural Uganda and the associated treatment patterns and delays in care. Methods: This is a retrospective cohort study of a trauma registry that was implemented at Masindi-Kitara Medical Center (MKMC), a rural hospital in Western Uganda. Demographic information, injury characteristics, modified Kampala Trauma Scores (M-KTS), and treatment modalities over a 12 month period were retrospectively collected from paper-based registry forms completed for all injury patients presenting to MKMC. Results: A total of 350 patients were entered into the trauma registry. Most patients were male (71.2%) with a median age of 26.5 years. Motorcycle crashes were the most prevalent mechanism of injury (42.3%) with the majority being unhelmeted (83.3%). Soft tissue injury was the most common diagnosis (44.9%). Patients were frequently treated in the outpatient department and then discharged (54.8%). Patients requiring admission or transfer (M-KTS = 11.57 or 11.67) tended to have a lower M-KTS than discharged patients (M-KTS = 12.75). Analgesics (74.6%) and antibiotics (52.9%) were the most common treatments administered. For those patients requiring admission (29.4%), only one in-hospital death was documented. Thirty-nine percent of patients reported a delay in seeking care, most frequently due to lack of transportation (31.5%) with a median time of delay of 11 h. Conclusion: Road traffic injuries were the leading cause of injury in Masindi, with a high proportion of injuries associated with unhelmeted motorcycle crashes. Future opportunities to prevent injury and improve care may be seen through improved prehospital care, enforcement of helmet laws, increased access to neurosurgical services, and enactment of hospital quality improvement measures