Novel Insights Into Gyrate Atrophy of the Choroid and Retina (GACR): A Cohort Study.

Gyrate atrophy of the choroid and retina (GACR, OMIM #258870) is a rare inherited metabolic disorder characterized by progressive chorioretinal degeneration and hyperornithinemia. Current therapeutic modalities potentially slow disease progression but are not successful in preventing blindness. To allow for trial development, increased knowledge of the clinical phenotype and current therapeutic outcomes is required.

Reliability and reproducibility of systematic reviews informing the 2020-2025 Dietary Guidelines for Americans: a pilot study.

Background: Although high-quality nutrition systematic reviews (SRs) are important for clinical decision making, there remains debate on their methodological quality and reporting transparency.

Objectives: The objective of this study was to assess the reliability and reproducibility of a sample of SRs produced by the Nutrition Evidence Systematic Review (NESR) team to inform the 2020-2025 Dietary Guidelines for Americans (DGAs).

Methods: We evaluated a sample of 8 SRs from the DGA dietary patterns subcommittee for methodological quality using the Assessment of Multiple Systematic Reviews 2 (AMSTAR 2) tool and for reporting transparency using the PRISMA 2020 and PRISMA literature search extension (PRISMA-S) checklists.

Aldolase B Deficient Mice Are Characterized by Hepatic Nucleotide Sugar Abnormalities.

Hereditary fructose intolerance (HFI) is characterized by liver damage and a secondary defect in N-linked glycosylation due to impairment of mannose phosphate isomerase (MPI). Mannose treatment has been shown to be an effective treatment in a primary defect in MPI (i.e.

Experiences and needs of residents with dementia in relocating to an innovative living arrangement within long-term care: A qualitative study.

During the last decade, an increasing number of care organizations have chosen to rebuild or build a new care facility to provide better person-environments for residents with dementia. This has inevitably led to an increase in relocations. This study investigated how residents with dementia experienced a relocation from a regular nursing home to an innovative living arrangement.

Fructose restriction has beneficial effects on adipose tissue distribution but not on serum adipokine levels: Post-hoc analysis of a double-blind randomized controlled trial.

We aimed to examine the effects of isocaloric fructose restriction on adipose tissue distribution and serum adipokines. Individuals with BMI >28 kg/m (n = 44) followed a 6-week fructose-restricted diet and were randomly allocated to (double-blind) oral supplementation with fructose (control) or glucose (intervention) powder three times daily. Visceral (VAT) and subcutaneous (SAT) adipose tissue was quantified with MRI.

A Review of Evidence Related to the Zoonotic Characteristics of the Monkeypox Virus.

The 2022 monkeypox virus (MPXV) outbreaks spurred global public health concern. In response, we undertook a living systematic review of its zoonotic characteristics, including potential reservoirs and susceptible species, transmissibility, and clinical presentation in nonhuman species. Electronic database searches yielded 148 eligible records published between 2000 and 2022.

Depressive Signs and Daily Life of Residents When Relocating from a Regular to an Innovative Nursing Home.

Objectives: In this study, we examine how residents are affected by moving from a regular nursing home into an innovative living arrangement. In the past decade, a culture change has taken place, leading to rapid developments of innovative living arrangements that aim to change the physical, social, and organizational environment to better suit the needs of older adults needing 24-hour care. This has inevitably led to more group relocations in long-term care.

How firearm legislation impacts firearm mortality internationally: A scoping review.

Background: The literature on gun violence is broad and variable, describing multiple legislation types and outcomes in observational studies. Our objective was to document the extent and nature of evidence on the impact of firearm legislation on mortality from firearm violence.

Methods: A scoping review was conducted under PRISMA-ScR guidance.

Fracture risk revisited: Bone mineral density T-score and fracture risk in type 2 diabetes.

Aim: To study the association between femoral neck (FN) bone mineral density (BMD) T-score and fracture risk in individuals with and without type 2 diabetes (T2D).

Materials And Methods: We performed a single-centre retrospective cohort study using the Danish National Health Service. BMD of the FN was measured by dual-energy X-ray absorptiometry.

The association of type 2 diabetes-related characteristics with fracture risk at different sites.

Aim: To determine the association of diabetes-related characteristics with fractures at different sites in individuals with type 2 diabetes (T2D).

Materials And Methods: We conducted a cohort study using the Clinical Practice Research Datalink (CPRD) GOLD. Patients aged over 30 years with T2D were identified within the CPRD.

Indocyanine green near-infrared fluorescence bowel perfusion assessment to prevent anastomotic leakage in minimally invasive colorectal surgery (AVOID): a multicentre, randomised, controlled, phase 3 trial.

Background: Anastomotic leakage is a severe postoperative complication in colorectal surgery and compromised bowel perfusion is considered a major contributing factor. Conventional methods to assess bowel perfusion have a low predictive value for anastomotic leakage. We therefore aimed to evaluate the efficacy of real-time assessment with near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) in the prevention of anastomotic leakage.

A Fulbright International Exchange to Expand the Physician Assistant/Associate Curriculum in Geriatrics.

The international growth of the physician assistant/associate (PA) profession provides opportunities for global exchange that PA faculty and clinicians should seek out and cultivate. Physician assistant/associate programs can benefit from the experiences and expertise of faculty educators from different backgrounds and countries. The Fulbright International Scholars Program is a well-established and recognized organization dedicated to supporting international exchange in higher education.

Association Between Low Sex Hormone-Binding Globulin and Increased Risk of Type 2 Diabetes Is Mediated by Increased Visceral and Liver Fat: Results From Observational and Mendelian Randomization Analyses.

The aim of this study was to investigate the associations among sex hormone-binding globulin (SHBG), visceral adipose tissue (VAT), liver fat content, and risk of type 2 diabetes (T2D). In the Netherlands Epidemiology of Obesity study, 5,690 women (53%) and men (47%) without preexisting diabetes were included and followed for incident T2D. SHBG concentrations were measured in all participants, VAT was measured using MRI, and liver fat content was measured using proton magnetic resonance spectroscopy in a random subset of 1,822 participants.

How does guided group reflection work to support professional identity formation in postgraduate medical education: A scoping review.

Purpose: In postgraduate medical education, guided group reflection is often applied to support professional identity formation. However, little is known about how guided group reflection is shaped and how it works. Our scoping review synthesizes existing evidence about various approaches for guided group reflection, their aims, components and potential working mechanisms.

One size doesn't fit all: methodological reflections in conducting community-based behavioural science research to tailor COVID-19 vaccination initiatives for public health priority populations.

Background: Promoting the uptake of vaccination for infectious diseases such as COVID-19 remains a global challenge, necessitating collaborative efforts between public health units (PHUs) and communities. Applied behavioural science can play a crucial role in supporting PHUs' response by providing insights into human behaviour and informing tailored strategies to enhance vaccination uptake. Community engagement can help broaden the reach of behavioural science research by involving a more diverse range of populations and ensuring that strategies better represent the needs of specific communities.

Brain function in classic galactosemia, a galactosemia network (GalNet) members review.

Classic galactosemia (CG, OMIM #230400, ORPHA: 79,239) is a hereditary disorder of galactose metabolism that, despite treatment with galactose restriction, affects brain function in 85% of the patients. Problems with cognitive function, neuropsychological/social emotional difficulties, neurological symptoms, and abnormalities in neuroimaging and electrophysiological assessments are frequently reported in this group of patients, with an enormous individual variability. In this review, we describe the role of impaired galactose metabolism on brain dysfunction based on state of the art knowledge.

Screening for depression in children and adolescents in primary care or non-mental health settings: a systematic review update.

Background: The transition from childhood to adolescence is associated with an increase in rates of some psychiatric disorders, including major depressive disorder, a debilitating mood disorder. The aim of this systematic review is to update the evidence on the benefits and harms of screening for depression in primary care and non-mental health clinic settings among children and adolescents.

Methods: This review is an update of a previous systematic review, for which the last search was conducted in 2017.

Fructose intake from sugar-sweetened beverages is associated with a greater risk of hyperandrogenism in women: UK Biobank cohort study.

Objective: To assess the association between fructose consumption and serum sex hormone-binding globulin (SHBG), (free) testosterone, and risk of hyperandrogenism in a population-based cohort.

Design: An observational and genetic association study in participants of the UK Biobank (n = 136 384 and n = 383 392, respectively).

Methods: We assessed the relationship of (1) the intake of different sources of fructose (ie, total, fruit, fruit juice, and sugar-sweetened beverages [SSBs]) and (2) rs2304681 (a missense variant in the gene encoding ketohexokinase, used as an instrument of impaired fructose metabolism), with SHBG, total and free testosterone levels, and risk of hyperandrogenism (free androgen index >4.

Screening for hypertension in adults: protocol for evidence reviews to inform a Canadian Task Force on Preventive Health Care guideline update.

Purpose: To inform updated recommendations by the Canadian Task Force on Preventive Health Care on screening in a primary care setting for hypertension in adults aged 18 years and older. This protocol outlines the scope and methods for a series of systematic reviews and one overview of reviews.

Methods: To evaluate the benefits and harms of screening for hypertension, the Task Force will rely on the relevant key questions from the 2021 United States Preventive Services Task Force systematic review.

A study protocol for implementing Canadian Practice Guidelines for Treating Children and Adolescents with Eating Disorders.

Background: Eating disorders have one of the highest mortality rates among psychiatric illnesses. Timely intervention is crucial for effective treatment, as eating disorders tend to be chronic and difficult to manage if left untreated. Clinical practice guidelines play a vital role in improving healthcare delivery, aiming to minimize variations in care and bridge the gap between research and practice.

Relationship between educational attainment and non-alcoholic fatty liver disease: A two-sample Mendelian randomization study.

Background: Observational studies have identified an inverse association between education and non-alcoholic fatty liver disease (NAFLD). However, it is not possible to establish causality for this relationship.

Aims: To gain more insight into the causal nature of the relationship between education and NAFLD.