Intraocular Pressure After Anti-Vascular Endothelial Growth Factor Injection in Eyes With a Mineralized Bruch's Membrane Caused by Pseudoxanthoma Elasticum.

Purpose: To assess acute IOP changes after anti-VEGF injections in patients with Pseudoxanthoma elasticum (PXE) compared to other retinal diseases.

Methods: Twenty eyes of patients with PXE (mean age 63.4 ± 6.

Accelerating the transition from a linear to a circular healthcare sector: ESCH-R: study design and methodology.

Due to the significant environmental impact of healthcare, there is an urgent need to accelerate its circular transition. We provide an overview of the ESCH-R project study design and methodology for accelerating the transition from a linear to a circular healthcare sector through the development and implementation of circular interventions in the Netherlands. Using a transdisciplinary approach, we will apply the 10-R ladder framework for a circular economy to hospitals.

Remote web-based self-assessment of visual acuity versus ETDRS in patients with macular diseases: a method comparison study.

Background: Macular diseases (MD) lead to frequent clinic visits, involve time-consuming visual acuity (VA) measurements by professionals. Independent home measurements could improve efficiency. This study evaluates the agreement of a web-based test in MD compared to in-hospital measurements.

[Retinal artery and vein occlusions].

Retinal artery and vein occlusions can both lead to significant vision loss. Vein occlusions are much more common, while visual acuity is usually more severely affected in artery occlusions. In addition to ophthalmological treatment, both types of occlusions require a multidisciplinary approach, in which cardiovascular risk management is important.

Blood integrin- and cytokine-producing T cells are associated with stage and genetic risk score in age-related macular degeneration.

Age-related macular degeneration (AMD) remains a leading cause of vision loss in the geriatric population. There are age-related changes in peripheral blood leukocyte composition, but their significance for AMD remains unclear. We aimed to determine changes in immune cell populations in the blood of AMD patients.

Assessing the Carbon Footprint of Telemedicine: A Systematic Review.

Background: Healthcare is responsible for 4% to 10% of carbon emissions worldwide, of which 22% is related to transport. Telemedicine emerged as a potential solution to reduce the footprint, for example, by reducing travel. However, a need to understand which variables to include in carbon footprint estimations in telemedicine limits our understanding of the beneficial impact telemedicine might have on our environment.

Pseudoxanthoma elasticum - Genetics, pathophysiology, and clinical presentation.

Pseudoxanthoma elasticum (PXE) is an autosomal-recessively inherited multisystem disease. Mutations in the ABCC6-gene are causative, coding for a transmembrane transporter mainly expressed in hepatocytes, which promotes the efflux of adenosine triphosphate (ATP). This results in low levels of plasma inorganic pyrophosphate (PPi), a critical anti-mineralization factor.

Bruch's Membrane Calcification in Pseudoxanthoma Elasticum: Comparing Histopathology and Clinical Imaging.

Purpose: To investigate the histology of Bruch's membrane (BM) calcification in pseudoxanthoma elasticum (PXE) and correlate this to clinical retinal imaging.

Design: Experimental study with clinicopathological correlation.

Subjects And Controls: Six postmortem eyes from 4 PXE patients and 1 comparison eye from an anonymous donor without PXE.

Plasma Level of Pyrophosphate Is Low in Pseudoxanthoma Elasticum Owing to Mutations in the ABCC6 Gene, but It Does Not Correlate with ABCC6 Genotype.

Background: Pseudoxanthoma elasticum (PXE), a monogenic disorder resulting in calcification affecting the skin, eyes and peripheral arteries, is caused by mutations in the ABCC6 gene, and is associated with low plasma inorganic pyrophosphate (PP). It is unknown how ABCC6 genotype affects plasma PP.

Methods: We studied the association of ABCC6 genotype (192 patients with biallelic pathogenic ABCC6 mutations) and PP levels, and its association with the severity of arterial and ophthalmological phenotypes.

A view from the clinic - Perspectives from Dutch patients and professionals on high myopia care.

Purpose: To understand and compare perspectives of patients and professionals on current ophthalmologic care for high myopia, and to identify challenges and future opportunities.

Methods: Self-reported data were collected through two online questionnaires. Patient perspective was obtained from highly myopic members of a patient organisation based in the Netherlands using a 17-item questionnaire consisting of open and multiple-choice questions regarding personal experience with myopia care.

What did we learn in 35 years of research on nutrition and supplements for age-related macular degeneration: a systematic review.

The aim of this paper is to summarize all available evidence from systematic reviews, randomized controlled trials (RCTs) and comparative nonrandomized studies (NRS) on the association between nutrition and antioxidant, vitamin, and mineral supplements and the development or progression of age-related macular degeneration (AMD). The Cochrane Database of Systematic Reviews, Cochrane register CENTRAL, MEDLINE and Embase were searched and studies published between January 2015 and May 2021 were included. The certainty of evidence was assessed according to the GRADE methodology.

Stargardt disease: monitoring incidence and diagnostic trends in the Netherlands using a nationwide disease registry.

Purpose: To assess the incidence of Stargardt disease (STGD1) and to evaluate demographics of incident cases.

Methods: For this retrospective cohort study, demographic, clinical and genetic data of patients with a clinical diagnosis of STGD1 were registered between September 2010 and January 2020 in a nationwide disease registry. Annual incidence (2014-2018) and point prevalence (2018) were assessed on the basis of this registry.

Genotype-phenotype correlation in pseudoxanthoma elasticum.

Background And Aims: Pseudoxanthoma elasticum (PXE) is caused by variants in the ABCC6 gene. It results in calcification in the skin, peripheral arteries and the eyes, but has considerable phenotypic variability. We investigated the association between the ABCC6 genotype and calcification and clinical phenotypes in these different organs.

The Natural History of Bruch's Membrane Calcification in Pseudoxanthoma Elasticum.

Purpose: To describe the natural history of Bruch's membrane (BM) calcification in patients with pseudoxanthoma elasticum (PXE).

Design: Retrospective cohort study.

Participants: Both eyes of 120 PXE patients younger than 50 years, 78 of whom had follow-up imaging after more than 1 year.

Association of Rhegmatogenous Retinal Detachment Incidence With Myopia Prevalence in the Netherlands.

Importance: The incidence of rhegmatogenous retinal detachment (RRD) is partly determined by its risk factors, such as age, sex, cataract surgery, and myopia. Changes in the prevalence of these risk factors could change RRD incidence in the population.

Objective: To determine whether the incidence of RRD in the Netherlands has changed over recent years and whether this change is associated with an altered prevalence of RRD risk factors.

The effect of etidronate on choroidal neovascular activity in patients with pseudoxanthoma elasticum.

Aim: To assess the effect of the bisphosphonate etidronate on choroidal neovascular (CNV) activity in patients with pseudoxanthoma elasticum (PXE).

Methods: This is an ancillary study in a single center, randomized, double-blind placebo-controlled trial (RCT) in which 74 patients with PXE were assigned to either one-year etidronate or placebo treatment. Spectral domain optical coherence tomography (SD-OCT) imaging and color fundus photography were performed every three months for one year and were systematically assessed on signs of CNV activity.

A Reflectivity Measure to Quantify Bruch's Membrane Calcification in Patients with Pseudoxanthoma Elasticum Using Optical Coherence Tomography.

Purpose: Progressive calcification of Bruch's membrane (BM) causes considerable visual morbidity in patients with pseudoxanthoma elasticum (PXE). Since calcification is hyperreflective on optical coherence tomography (OCT), our aim was to measure BM calcification with OCT imaging.

Methods: Case-control study with 45 patients with PXE under 40 years (range, 11-39) and 25 controls (range, 14-39).

The Extent of Angioid Streaks Correlates With Macular Degeneration in Pseudoxanthoma Elasticum.

Purpose: To investigate whether the extent of Bruch's membrane calcification is associated with choroidal neovascularization (CNV) and macular atrophy in patients with pseudoxanthoma elasticum (PXE) by using the extent of angioid streaks as a surrogate marker for the degree of Bruch's membrane calcification.

Design: Retrospective cross-sectional study.

Methods: We investigated 301 patients with PXE (median age, 52 years; range, 9-79 years) in a tertiary referral center.

Comparing the Efficacy of Bevacizumab and Ranibizumab in Patients with Diabetic Macular Edema (BRDME): The BRDME Study, a Randomized Trial.

Purpose: To generate conclusive evidence regarding the noninferiority of intravitreal bevacizumab compared with ranibizumab in patients with diabetic macular edema (DME).

Design: Comparative, randomized, double-masked, multicenter, noninferiority clinical trial.

Participants: Eligible patients were older than 18 years, diagnosed with type 1 or type 2 diabetes mellitus, with glycosylated hemoglobin of less than 12%, central area thickness of more than 325 μm, and visual impairment from DME with a best-corrected visual acuity (BCVA) between 24 letters and 78 letters.

IS VAGINAL DELIVERY HARMFUL TO PATIENTS WITH PSEUDOXANTHOMA ELASTICUM?

Background/purpose: To investigate the effect of a vaginal delivery (VD) on retinal pathology in patients with pseudoxanthoma elasticum.

Methods: Retrospective case series. All 14 consecutive women with pseudoxanthoma elasticum who visited the ophthalmology department during pregnancy and after delivery between 2010 and 2018 were included.

Comparing the Efficacy of Bevacizumab and Ranibizumab in Patients with Retinal Vein Occlusion: The Bevacizumab to Ranibizumab in Retinal Vein Occlusions (BRVO) study, a Randomized Trial.

Purpose: Comparing the efficacy of intravitreal injections of bevacizumab to ranibizumab in the treatment of macular edema (ME) resulting from retinal vein occlusion (RVO).

Design: Comparative, randomized, double-masked, multicenter, noninferiority clinical trial. The noninferiority margin was 4 letters.

Is arterial stiffness in the carotid artery associated with choroidal thinning in patients with pseudoxanthoma elasticum or controls?

Purpose: Patients with pseudoxanthoma elasticum (PXE) develop calcification of Bruch's membrane (BM) and choroidal thinning, as well as calcification of intracranial arteries, leading to arterial stiffness. We investigated whether arterial stiffness is associated with choroidal thinning in PXE patients, besides the presumed effect of BM calcification.

Methods: Cross-sectional study with 75 PXE patients and 40 controls.

Risk of Rhegmatogenous Retinal Detachment in Acute Retinal Necrosis With and Without Prophylactic Intervention.

Purpose: To study whether preventive laser or preventive vitrectomy is able to lower the risk of rhegmatogenous retinal detachment (RRD) in patients with acute retinal necrosis (ARN).

Design: A retrospective, interventional case series.

Methods: We performed a retrospective study of 59 patients (63 eyes) with ARN treated in a single tertiary referral center.