Leber Congenital Amaurosis Due to GUCY2D Mutations: Longitudinal Analysis of Retinal Structure and Visual Function

Gene augmentation therapy is being planned for GUCY2D-associated Leber congenital amaurosis (LCA). To increase our understanding of the natural history of GUCY2D-LCA, patients were evaluated twice with an interval of 4 to 7 years between visits using safety and efficacy outcome measures previously determined to be useful for monitoring this disorder. In this group of molecularly-identified LCA patients (n = 10; ages 7–37 years at first visit), optical coherence tomography (OCT) was used to measure foveal cone outer nuclear layer (ONL) thickness and rod ONL at a superior retinal locus. Full-field stimulus testing (FST) with chromatic stimuli in dark- and light-adapted states was used to assay rod and cone vision. Changes in OCT and FST over the interval were mostly attributable to inter-visit variability. There were no major negative changes in structure or function across the cohort and over the intervals studied. Variation in severity of disease expression between patients occurs; however, despite difficulties in quantifying structure and function in such seriously visually impaired individuals with nystagmus, the present work supports the use of OCT as a safety outcome and FST as an efficacy outcome in a clinical trial of GUCY2D-LCA. A wide age spectrum for therapy was confirmed, and there was relative stability of structure and function during a typical time interval for clinical trials.     

Caenorhabditis elegans as a Model System for Duchenne Muscular Dystrophy

The nematode worm Caenorhabditis elegans has been used extensively to enhance our understanding of the human neuromuscular disorder Duchenne Muscular Dystrophy (DMD). With new arising clinically relevant models, technologies and treatments, there is a need to reconcile the literature and collate the key findings associated with this model.     

Using reduced-processing training to improve decision efficiency among perfectionists

Cognition, Technology & Work - Previous research has demonstrated performance inefficiencies among perfectionists, which may be mitigated via the adoption of ‘good enough’...     

Manure Management at Ohio Confined Animal Feeding Facilities in the Maumee River Watershed

In 2015, 48 permitted Confined Animal Feeding Operations (CAFOs) housed approximately 90% of poultry and 20% of swine and cattle within the Ohio portion of the Maumee River watershed. Recently, concerns about the impact CAFOs may have on nutrient loading in the watershed have been raised. In this study, we used manure management plans and inspection reports obtained from the Ohio Department of Agriculture Division of Livestock Environmental Permitting (ODA-DLEP) to assess how these CAFOs managed their manure for the years 2014 and 2015. A majority of liquid manure was applied between April and October, closely matching the amount of liquid manure planned to be applied during this period. Approximately 79% of the acres under control of the CAFOs that received manure had Bray P1 soil test phosphorus values below 50 ppm. The average distance between a swine CAFO’s livestock holding barn to the fields they control that can receive manure was 1.43 miles while for cattle CAFOs this distance was 1.91 miles. Approximately 78% of manure phosphorus generated on CAFOs was planned to be transferred through Distribution and Utilization, a process in which ownership of manure changes hands, including virtually all solid poultry manure phosphorus. While publicly available data show that, in general, CAFOs in the region are adhering to their state-approved permits, a knowledge gap regarding the management of approximately 80% of manure phosphorus exists due to manure transferred through Distribution and Utilization and manure produced from non-permitted livestock operations.     

A home among the gum trees: the Victorian Sanatorium for consumptives,Echuca and Mount Macedon

The history of the treatment of tuberculosis in Australia has largely been focused on the development of medical treatments, the architectural features of custom-built sanatoria and the human impact of the disease in the nineteenth and early twentieth centuries. These discussions often mention contemporary debates amongst medical men and the laity regarding the best treatment, but the influence of medical climatology in deciding where medical facilities should be placed is often overlooked. The first sanatorium in the Colony of Victoria had two branches: Echuca and Mount Macedon. These two locations differ in terms of altitude, the surrounding forest and meteorological variation. Yet, both sites were considered suitable at the time for the location of a sanatorium, possibly due to the health-promoting and aesthetic aspects of nearby eucalypt forests. This article explores why they may at first modernly appear to be substantially different locations, but contemporary medical climatology emphasised their similarities.     

Occurrence of rotaviruses and enteroviruses in recreational waters of Oak Creek, Arizona

Recent epidemiological studies have shown a relationship between swimming in recreational waters meeting bacteriological standards and gastroenteritis with a suggested viral etiology. No previous studies have been conducted in the United States on the occurrence of human pathogenic enteric viruses in freshwater recreational areas. The presence of enteroviruses and rotaviruses was investigated in Oak Creek, Arizona, a heavily used recreational area. Water samples were filtered through positively charged filters (168–1555 I.), eluted with beef extract, and assayed for human enteroviruses and rotaviruses. Eighteen of the 41 recreational water samples were positive for enterovirus or rotavirus. Of these, nine samples exceeded the Arizona State recommended limit of 1 PFU 40 l⁻¹ for full body contact in effluent dominated recreational waters. Several virus positive samples met the recommended fecal coliform standards (200 CFU 100 ml⁻¹) for recreational waters indicating the inadequacy of bacterial standards for monitoring viral water quality. The isolation of the pathogenic enteric viruses (i.e., poliovirus 1, echovirus 1, coxsackievirus B1 and B6 and rotavirus) from this popular recreational water demonstrates the potential for transmission of viral disease.