Bilateral Proliferative Retinopathy as the Initial Presentation of Atypical Hemolytic Uremic Syndrome: A Case Report.

In this case report, we describe a 34-year-old male patient who presented with vision loss and was found to have profound occlusive retinal vasculopathy. His initial laboratory studies were unremarkable, but five weeks after his ocular symptoms began, he developed acute multi-organ failure and was ultimately diagnosed with atypical hemolytic uremic syndrome (aHUS). His course was complicated by a stroke, respiratory distress requiring intubation, long-term hemodialysis, and eventually death. Occlusive retinal vasculopathy may be the presenting finding in aHUS, although thrombotic microangiopathy syndromes typically present with acute kidney injury and or failure, hemolytic anemia, and thrombocytopenia. [Ophthalmic Surg Lasers Imaging Retina 2023;54:297-300.].

Objective evaluation of vision quality in pseudophakic patients with posterior capsular opacification using double-pass retinal imaging / Avaliação objetiva da qualidade da visão em pacientes pseudofácicos com opacificação capsular posterior pelo método de dupla passagem de luz

ABSTRACT Purpose: The purpose of this study was to objectively assess the optical vision quality of patients before and after Nd:YAG capsulotomy for posterior capsular opacification using a double-pass retinal imaging system. Methods: We retrospectively analyzed the data from 26 pseudophakic eyes with posterior capsular opacification that underwent Nd:YAG capsulotomy. The objective scatter indices, modulation transfer function cutoff frequencies, Strehl ratios, and logMAR corrected distance visual acuities were assessed before and after YAG capsulotomy with a double-pass retinal imaging system (OQAS II, Visiometrics, Spain). We also analyzed data from a subgroup of 10 patients with pre-YAG corrected distance visual acuity of 0.10 logMAR (20/25) or better. Results: Vision quality indices improved in all 26 eyes, resulting in a statistically significant improvement in the corrected distance visual acuity (p=0.007), objective scatter index (p=0.001), modulation transfer function cutoff frequency (p=0.001), and Strehl ratio (p=0.020). The overall mean improvements included 0.12 ± 0.04 logMAR for corrected distance visual acuity, 2.84 ± 0.76 for objective scatter index, 12.29 ± 2.77 for modulation transfer function cutoff frequency, and 0.06 ± 0.03 for Strehl ratio. Our sub-analysis of 10 eyes with 0.10 logMAR (20/25) corrected distance visual acuity or better also showed a statistically significant improvement in the mean objective scatter index (0.76 ± 16; p=0.001), resulting in approximately 35% decrease in intraocular light scatter. Conclusions: The objective vision quality measurements as assessed by the double-pass retinal imaging system showed a significant improvement after YAG capsulotomy. This suggests that the objective scatter index improves after YAG capsulotomy, even in eyes with pre-YAG 0.10 logMAR (Snellen 20/25) corrected distance visual acuity or better.

RESUMO Objetivo: Avaliar objetivamente a qualidade da visão óptica antes e depois da capsulotomia com Nd: YAG, obtida por imagem pelo metodo de dupla passagem de luz em pacientes com opacificação capsular posterior. Método: Análise retrospectiva de 26 olhos pseudofácicos com opacificação capsular posterior visualmente significativa, que foram submetidos à capsulotomia de Nd: YAG. O índice de dispersão objetiva, a função de transferência de modulação, a relação de Strehl e a acuidade visual à distância corrigida foram avaliados antes e após a capsulotomia com YAG usando o dupla passagem de luz (OQAS II, Visiometrics, Espanha). Também foi analisado um subgrupo de pacientes com acuidade visual à distância corrigida pré-YAG de 0,10 logMAR (20/25) ou melhor. Resultados: Os índices de qualidade da visão melhoraram em todos os 26 olhos, resultando em uma melhora estatisticamente significativa na acuidade visual à distância corrigida (p=0,007), índice de dispersão objetiva (p=0,001), função de transferência de modulação (p=0,001) e relação de Strehl (p=0,020). A melhora média na acuidade visual à distância corrigida foi de 0,12 ± 0,04 logMAR, no índice de dispersão objetiva foi de 2,84 ± 0,76, no função de transferência de modulação foi de 12,29 ± 2,77 e na razão de Strehl foi de 0,06 ± 0,03 em todos os olhos. Sub-análise de 10 olhos com 0.10 logMAR (20/25) acuidade visual à distância corrigida ou melhor também mostrou uma melhora estatisticamente significativa no índice de dispersão objetiva (p=0,001). A melhora média no OSI foi de 0,76 ± 16, uma diminuição de 35% na dispersão da luz intraocular. Conclusões: A qualidade da visão avaliada pelo dupla passagem de luz mostra uma melhora significativa nas medidas objetivas da qualidade da visão após a capsulotomia do YAG. O índice de dispersão objetiva melhora após a capsulotomia YAG, mesmo em olhos com acuidade visual à distância corrigida pré-YAG de 0,10 logMAR (Snellen 20/25) ou melhor.

Objective evaluation of vision quality in pseudophakic patients with posterior capsular opacification using double-pass retinal imaging.

PURPOSE: The purpose of this study was to objectively assess the optical vision quality of patients before and after Nd:YAG capsulotomy for posterior capsular opacification using a double-pass retinal imaging system. METHODS: We retrospectively analyzed the data from 26 pseudophakic eyes with posterior capsular opacification that underwent Nd:YAG capsulotomy. The objective scatter indices, modulation transfer function cutoff frequencies, Strehl ratios, and logMAR corrected distance visual acuities were assessed before and after YAG capsulotomy with a double-pass retinal imaging system (OQAS II, Visiometrics, Spain). We also analyzed data from a subgroup of 10 patients with pre-YAG corrected distance visual acuity of 0.10 logMAR (20/25) or better. RESULTS: Vision quality indices improved in all 26 eyes, resulting in a statistically significant improvement in the corrected distance visual acuity (p=0.007), objective scatter index (p=0.001), modulation transfer function cutoff frequency (p=0.001), and Strehl ratio (p=0.020). The overall mean improvements included 0.12 ± 0.04 logMAR for corrected distance visual acuity, 2.84 ± 0.76 for objective scatter index, 12.29 ± 2.77 for modulation transfer function cutoff frequency, and 0.06 ± 0.03 for Strehl ratio. Our sub-analysis of 10 eyes with 0.10 logMAR (20/25) corrected distance visual acuity or better also showed a statistically significant improvement in the mean objective scatter index (0.76 ± 16; p=0.001), resulting in approximately 35% decrease in intraocular light scatter. CONCLUSIONS: The objective vision quality measurements as assessed by the double-pass retinal imaging system showed a significant improvement after YAG capsulotomy. This suggests that the objective scatter index improves after YAG capsulotomy, even in eyes with pre-YAG 0.10 logMAR (Snellen 20/25) corrected distance visual acuity or better.

Targets for AD treatment: conflicting messages from γ-secretase inhibitors.

Current evidence suggests that Alzheimer's disease (AD) is a multi-factorial disease that starts with accumulation of multiple proteins. We have previously proposed that inhibition of γ-secretase may impair membrane recycling causing neurodegeneration starting at synapses (Sambamurti K., Suram A., Venugopal C., Prakasam A., Zhou Y., Lahiri D. K. and Greig N. H. A partial failure of membrane protein turnover may cause Alzheimer's disease: a new hypothesis. Curr. Alzheimer Res., 3, 2006, 81). We also proposed familal AD mutations increase Aß42 by inhibiting γ-secretase. Herein, we discuss the failure of Eli Lilly's γ-secretase inhibitor, semagacestat, in clinical trials in the light of our hypothesis, which extends the problem beyond toxicity of Aß aggregates. We elaborate that γ-secretase inhibitors lead to accumulation of amyloid precursor protein C-terminal fragments that can later be processed by γ-secretase to yields bursts of Aß to facilitate aggregation. Although we do not exclude a role for toxic Aß aggregates, inhibition of γ-secretase can affect numerous substrates other than amyloid precursor protein to affect multiple pathways and the combined accumulation of multiple peptides in the membrane may impair its function and turnover. Taken together, protein processing and turnover pathways play an important role in maintaining cellular homeostasis and unless we clearly see consistent disease-related increase in their levels or activity, we need to focus on preserving their function rather than inhibiting them for treatment of AD and similar diseases.