Surgery for equinovarus deformity in Friedreich's ataxia improves mobility and independence

Friedreich's ataxia results in morbidity because of many factors; progressive equinovarus deformity is one of these. We studied the risk factors and incidence of this deformity. We sought to assess whether surgical management of fixed equinovarus deformity leads to functional improvement. Thirty-six patients with Friedrich's ataxia were assessed for this deformity. These patients were treated by splinting, botulinum toxin Type A injection, and surgery, as indicated by the severity, followed by an ongoing rehabilitation program. The effect of surgery was assessed using subscales of the Barthel index and functional independence measure. Severe foot deformities in which either surgery or botulinum toxin injection was recommended correlated with current age, years since disease onset, and years that the patient required a wheelchair for mobility, but not with the GAA repeat size or age at disease onset. Function and mobility were improved after surgery compared with a similar period before surgery. Three of seven patients who had surgery had significant complications. Aggressive management of foot deformities should be considered, and active measures to prevent permanent foot deformities should be pursued to maximize quality of life and independence of patients with Friedreich's ataxia.Level of Evidence: Therapeutic study, Level IV (case series-no, or historical control group).     

Influences of age and gender on workers' goals for retirement

Having clear goals for retirement is a critical determinant of life satisfaction and adjustment during the post-employment transition period. The purpose of the present study was to explore individuals' goals for retirement and determine whether age and gender differences exist among those goals. A sample of 55 working adults (aged 20-67) were asked to list their retirement goals. Items contained on respondents' lists were then classified into a taxonomy of retirement goals adapted from the work of LaPierre, Bastin, and Bouffard (1997). Both age and gender differences were identified in the concreteness of individuals' goals. Additionally, the number of retirement goals individuals hold decreased across the lifespan. Results also revealed patterns of age and gender differences across the various coding categories in the goal taxonomy. Findings are discussed in terms of the applied benefits of understanding the retirement goals of American workers.     

Retailer-Led Sugar-Sweetened Beverage Price Increase Reduces Purchases in a Hospital Convenience Store in Melbourne,Australia: A Mixed Methods Evaluation

Background

Limited evidence has been gathered on the real-world impact of sugar-sweetened beverage price changes on purchasing behavior over time or in community-retail settings.

The struggle for iron: gastrointestinal microbes modulate the host immune response during infection

The gastrointestinal track is one source of potential bacterial entry into the host, and the local immune system at the mucosal border is paramount in establishing host immune tolerance and the immune response to invading organisms. Macrophages use iron for production of hydroxy-radical and superoxide reactions, which are necessary for microbial killing. Presumably, as a survival strategy, bacteria, which also require iron for survival, have adapted the ability to sequester iron from the host, thereby limiting the availability to macrophages. As current modes of antimicrobial therapy are evolving, examination of nontraditional therapies is emerging. One such potential therapy involves altering the bacterial micronutrient iron concentration. Necrotizing enterocolitis is a clinical condition where such a strategy makes intuitive sense. This review will describe the immune response to gastrointestinal infection, the mechanisms that the gastrointestinal system uses to absorb intraluminal iron, and the critical role iron plays in the infectious process.     

Endoscopic management of polyps in a urinary continent catheterizable channel

We report a case of polyps in a urinary continent catheterizable channel that were endoscopically resected with the holmium laser. An 11-year-old boy underwent lower urinary tract reconstruction, along with a continent catheterizable channel. Due to persistent problems catheterizing, he underwent an endoscopy which revealed multiple broad-based polyps in his channel. These polyps were resected completely with the holmium laser without complication. The polyps were benign on final pathological examination. Follow-up endoscopy 4 weeks later revealed a healthy channel with no residual polyps. The patient has had no difficulties catheterizing in the interim. To our knowledge, this is the only report published of using the holmium laser to resect polyps in a urinary catheterizable channel.     

The significance of neuronal and glial cell changes in the rat retina during oxygen-induced retinopathy

Retinopathy of prematurity is a devastating vascular disease of premature infants. A number of studies indicate that retinal function is affected in this disease. Using the rat model of oxygen-induced retinopathy, it is possible to explore more fully the complex relationship between neuronal, glial and vascular pathology in this condition. This review examines the structural and functional changes that occur in the rat retina following oxygen-induced retinopathy. We highlight that vascular pathology in rats is characterized by aberrant growth of blood vessels into the vitreous at the expense of blood vessel growth into the body of the retina. Moreover, amino acid neurochemistry, a tool for examining neuronal changes in a spatially complete manner reveals widespread changes in amacrine and bipolar cells. In addition, neurochemical anomalies within inner retinal neurons are highly correlated with the absence of retinal vessels. The key cell types that link blood flow with neuronal function are macroglia. Macroglia cells, which in the retina include astrocytes and Müller cells, are affected by oxygen-induced retinopathy. Astrocyte loss occurs in the peripheral retina, while Müller cells show signs of reactive gliosis that is highly localized to regions that are devoid of intraretinal blood vessels. Finally, we propose that treatments, such as blockade of the renin–angiotensin system, that not only targets pathological angiogenesis, but that also promotes re-vascularization of the retina are likely to prove important in the treatment of those with retinopathy of prematurity.     

The renin–angiotensin system in retinal health and disease: Its influence on neurons,glia and the vasculature

Renin–Angiotensin System is classically recognized for its role in the control of systemic blood pressure. However, the retina is recognized to have all the components necessary for angiotensin II formation, suggestive of a role for Angiotensin II in the retina that is independent of the systemic circulation. The most well described effects of Angiotensin II are on the retinal vasculature, with roles in vasoconstriction and angiogenesis. However, it is now emerging that Angiotensin II has roles in modulation of retinal function, possibly in regulating GABAergic amacrine cells. In addition, Angiotensin II is likely to have effects on glia. Angiotensin II has also been implicated in retinal vascular diseases such as Retinopathy of Prematurity and diabetic retinopathty, and more recently actions in choroidal neovascularizaiton and glaucoma have also emerged. The mechanisms by which Angiotensin II promotes angiogensis in retinal vascular diseases is indicative of the complexity of the RAS and the variety of cell types that it effects. Indeed, these diseases are not purely characterized by direct effects of Angiotensin II on the vasculature. In retinopathy of prematurity, for example, blockade of AT₁ receptors prevents pathological angiogenesis, but also promotes revascularization of avascular regions of the retina. The primary site of action of Angiotensin II in this disease may be on retinal glia, rather than the vasculature. Indeed, blockade of AT₁ receptors prevents glial loss and promotes the re-establishment of normal vessel growth. Blockade of RAS as a treatment for preventing the incidence and progression of diabetic retinopathy has also emerged based on a series of studies in animal models showing that blockade of the RAS prevents the development of a variety of vascular and neuronal deficits in this disease. Importantly these effects may be independent of actions on systemic blood pressure. This has culminated recently with the completion of several large multi-centre clinical trials that showed that blockade of the RAS may be of benefit in some at risk patients with diabetes. With the emergence of novel compounds targeting different aspects of the RAS even more effective ways of blocking the RAS may be possible in the future.     

Generation, identification and functional characterization of the nob4 mutation of Grm6 in the mouse

We performed genome-wide chemical mutagenesis of C57BL/6J mice using N-ethyl-N-nitrosourea (ENU). Electroretinographic screening of the third generation offspring revealed two G3 individuals from one G1 family with a normal a-wave but lacking the b-wave that we named nob4. The mutation was transmitted with a recessive mode of inheritance and mapped to chromosome 11 in a region containing the Grm6 gene, which encodes a metabotropic glutamate receptor protein, mGluR6. Sequencing confirmed a single nucleotide substitution from T to C in the Grm6 gene. The mutation is predicted to result in substitution of Pro for Ser at position 185 within the extracellular, ligand-binding domain and oocytes expressing the homologous mutation in mGluR6 did not display robust glutamate-induced currents. Retinal mRNA levels for Grm6 were not significantly reduced, but no immunoreactivity for mGluR6 protein was found. Histological and fundus evaluations of nob4 showed normal retinal morphology. In contrast, the mutation has severe consequences for visual function. In nob4 mice, fewer retinal ganglion cells (RGCs) responded to the onset (ON) of a bright full field stimulus. When ON responses could be evoked, their onset was significantly delayed. Visual acuity and contrast sensitivity, measured with optomotor responses, were reduced under both photopic and scotopic conditions. This mutant will be useful because its phenotype is similar to that of human patients with congenital stationary night blindness and will provide a tool for understanding retinal circuitry and the role of ganglion cell encoding of visual information.     

Longitudinal Increases in Cerebral Brain Activation During Working Memory Performance in Friedreich Ataxia: 24-Month Data from IMAGE-FRDA

The Cerebellum - Friedreich ataxia (FRDA) has been associated with functional abnormalities in cerebral and cerebellar networks, particularly in the ventral attention network. However, how...     

The Nature and Extent of Online Marketing by Big Food and Big Alcohol During the COVID-19 Pandemic in Australia: Content Analysis Study

BackgroundEmerging evidence demonstrates that obesity is associated with a higher risk of COVID-19 morbidity and mortality. Excessive alcohol consumption and “comfort eating” as coping mechanisms during times of high stress have been shown to further exacerbate mental and physical ill-health. Global examples suggest that unhealthy food and alcohol brands and companies are using the COVID-19 pandemic to further market their products. However, there has been no systematic, in-depth analysis of how “Big Food” and “Big Alcohol” are capitalizing on the COVID-19 pandemic to market their products and brands.ObjectiveWe aimed to quantify the extent and nature of online marketing by alcohol and unhealthy food and beverage companies during the COVID-19 pandemic in Australia.MethodsWe conducted a content analysis of all COVID-19-related social media posts made by leading alcohol and unhealthy food and beverage brands (n=42) and their parent companies (n=12) over a 4-month period (February to May 2020) during the COVID-19 pandemic in Australia.ResultsNearly 80% of included brands and all parent companies posted content related to COVID-19 during the 4-month period. Quick service restaurants (QSRs), food and alcohol delivery companies, alcohol brands, and bottle shops were the most active in posting COVID-19-related content. The most common themes for COVID-19-related marketing were isolation activities and community support. Promotion of hygiene and home delivery was also common, particularly for QSRs and alcohol and food delivery companies. Parent companies were more likely to post about corporate social responsibility (CSR) initiatives, such as donations of money and products, and to offer health advice.ConclusionsThis is the first study to show that Big Food and Big Alcohol are incessantly marketing their products and brands on social media platforms using themes related to COVID-19, such as isolation activities and community support. Parent companies are frequently posting about CSR initiatives, such as donations of money and products, thereby creating a fertile environment to loosen current regulation or resist further industry regulation. “COVID-washing” by large alcohol brands, food and beverage brands, and their parent companies is both common and concerning. The need for comprehensive regulations to restrict unhealthy food and alcohol marketing, as recommended by the World Health Organization, is particularly acute in the COVID-19 context and is urgently required to “build back better” in a post-COVID-19 world.