A Retrospective Longitudinal Study of 460 Patients with ABCA4-Associated Retinal Disease.

Purpose: To investigate the distribution of genotypes and natural history of ABCA4-associated retinal disease in a large cohort of patients seen at a single institution.

Design: Retrospective, single-institution cohort review.

Participants: Patients seen at the University of Iowa between November 1986 and August 2022 clinically suspected to have disease caused by sequence variations in ABCA4.

Long-term daily stream temperature record for Scotland reveals spatio-temporal patterns in warming of rivers in the past and further warming in the future.

Stream temperature is directly and indirectly affected by climate change. To be able to project future changes in stream temperature, historic trends and factors influencing these trends need to be understood. There is a demand for daily data to analyse historical trends and future changes in stream temperature.

Evaluation and management consideration for children who are visually impaired.

Clinical low vision evaluations provided by optometrists or ophthalmologists trained and experienced in low vision rehabilitation care should be recommended for all children with a visual impairment, regardless of the cause of their vision loss, their age or the severity of their additional disabilities. This article discusses an effective model for pediatric vision rehabilitation service.

Embankment lowering and natural self-recovery improves river-floodplain hydro-geomorphic connectivity of a gravel bed river.

Floodplain reconnection can potentially improve the hydrogeomorphology of river corridors and attenuate flood peaks. However, empirical evidence of its effectiveness - needed to inform future restoration - is limited. A 70 m long flood embankment was lowered on the upper River Dee, a medium sized gravel bed river in north-east Scotland to reconnect a backwater and floodplain.

Predominance of hyperopia in autosomal dominant Best vitelliform macular dystrophy.

Background/aims: Patients with -associated autosomal dominant Best vitelliform macular dystrophy (AD-BVMD) have been reported to be hyperopic, but the prevalence of refractive error has not been described. This study aimed to characterise the type and degree of refractive error in a large cohort of patients with AD-BVMD compared with an age-similar group with -associated Stargardt disease.

Methods: This was a retrospective chart review of consecutive patients with molecularly confirmed AD-BVMD and Stargardt macular dystrophy seen at a single academic centre.

Attaining functional levels of visual acuity after vitrectomy for retinal detachment secondary to proliferative diabetic retinopathy.

Most patients needing diabetic tractional retinal detachment (TRD) surgery are working-age adults that drive and participate in other vision-dependent activities of daily living. We sought to determine the proportion of patients that achieve functional visual acuity (VA) based on the World Health Organization (WHO) definition of 'low vision' (≤ 20/80) and US driving standards (≥ 20/40) after vitrectomy for diabetic TRD. In this 10-year retrospective review, consecutive patients who underwent primary vitrectomy for TRD from proliferative diabetic retinopathy were studied.

Structure from motion photogrammetry in ecology: Does the choice of software matter?

Image-based modeling, and more precisely, Structure from Motion (SfM) and Multi-View Stereo (MVS), is emerging as a flexible, self-service, remote sensing tool for generating fine-grained digital surface models (DSMs) in the Earth sciences and ecology. However, drone-based SfM + MVS applications have developed at a rapid pace over the past decade and there are now many software options available for data processing. Consequently, understanding of reproducibility issues caused by variations in software choice and their influence on data quality is relatively poorly understood.

Auditory Global Positioning System and Advanced Driver Assistance Systems: A Safer Alternative to Bioptic Telescopes for Drivers Who Are Visually Impaired?

This work challenges the standard of the past 40 years, which required the use of a bioptic telescope by individuals with vision loss wanting to be licensed to drive in most states in the United States.Driving continues to be the key to independence for many individuals, particularly older drivers who live in an area where public transportation is limited or nonexistent. For the past 40 years, the most frequently option to allow drivers who are visually impaired to maintain driving privileges was to require them to use a bioptic telescope.

Integration of juvenile habitat quality and river connectivity models to understand and prioritise the management of barriers for Atlantic salmon populations across spatial scales.

Diadromous fish populations are strongly affected by in-stream barriers that cause river network fragmentation, constraining productivity or preventing completion of their lifecycle. Removal or reduction of barrier impacts is a restoration measure associated with unambiguous benefits. Management of barriers is therefore often prioritised above other restoration actions.

Low vision rehabilitation: An update.

This article provides information concerning issues related to the care of individuals who are visually impaired. Issues reviewed include determining who should be referred for vision rehabilitation services, Charles Bonnet syndrome, visual acuity, contrast sensitivity and visual field testing along with Useful Field of View testing. This article also discusses technology advances that can enhance the visual functioning of individuals who are visually impaired, including how these advances can help drivers with visual impairments to continue to safely operate motor vehicles, at least on a limited basis.

The science, policy and practice of nature-based solutions: An interdisciplinary perspective.

In this paper, we reflect on the implications for science, policy and practice of the recently introduced concept of Nature-Based Solutions (NBS), with a focus on the European context. First, we analyse NBS in relation to similar concepts, and reflect on its relationship to sustainability as an overarching framework. From this, we derive a set of questions to be addressed and propose a general framework for how these might be addressed in NBS projects by funders, researchers, policy-makers and practitioners.

Peroxisome biogenesis disorders in the Zellweger spectrum: An overview of current diagnosis, clinical manifestations, and treatment guidelines.

Peroxisome biogenesis disorders in the Zellweger spectrum (PBD-ZSD) are a heterogeneous group of genetic disorders caused by mutations in PEX genes responsible for normal peroxisome assembly and functions. As a result of impaired peroxisomal activities, individuals with PBD-ZSD can manifest a complex spectrum of clinical phenotypes that typically result in shortened life spans. The extreme variability in disease manifestation ranging from onset of profound neurologic symptoms in newborns to progressive degenerative disease in adults presents practical challenges in disease diagnosis and medical management.

Vision and driving: the United States.

Minimal visual standards for obtaining driving licensure in the United States principally use 2 measures: visual acuity and visual field. Although research studies have established a correlation between performance on these measures and safe driving, the correlations are weak and mostly retrospective. These measures remain in place in screening centers largely because they (especially visual acuity) are practical.

Vision and driving in multiple sclerosis.

Objective: To examine the relationship between measures of visual dysfunction and driving performance in persons with multiple sclerosis (MS).

Design: Between-group comparison.

Setting: All data were collected in an outpatient research setting.

Low vision rehabilitation: a concise overview.

This article provides a concise overview of low vision, evaluation and rehabilitation.