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BACKGROUNDIn retinitis pigmentosa (RP), rod photoreceptors degenerate from 1 of many mutations, after which cones are compromised by oxidative stress. N-acetylcysteine (NAC) reduces oxidative damage and increases cone function/survival in RP models. We tested the safety, tolerability, and visual function effects of oral NAC in RP patients.METHODSSubjects (n = 10 per cohort) received 600 mg (cohort 1), 1200 mg (cohort 2), or 1800 mg (cohort 3) NAC bid for 12 weeks and then tid for 12 weeks. Best-corrected visual acuity (BCVA), macular sensitivity, ellipsoid zone (EZ) width, and aqueous NAC were measured. Linear mixed-effects models were used to estimate the rates of changes during the treatment period.RESULTSThere were 9 drug-related gastrointestinal adverse events that resolved spontaneously or with dose reduction (maximum tolerated dose 1800 mg bid). During the 24-week treatment period, mean BCVA significantly improved at 0.4 (95% CI: 0.2-0.6, P < 0.001), 0.5 (95% CI: 0.3-0.7, P < 0.001), and 0.2 (95% CI: 0.02-0.4, P = 0.03) letters/month in cohorts 1, 2, and 3, respectively. There was no significant improvement in mean sensitivity over time in cohorts 1 and 2, but there was in cohort 3 (0.15 dB/month, 95% CI: 0.04-0.26). There was no significant change in mean EZ width in any cohort.CONCLUSIONOral NAC is safe and well tolerated in patients with moderately advanced RP and may improve suboptimally functioning macular cones. A randomized, placebo-controlled trial is needed to determine if oral NAC can provide long-term stabilization and/or improvement in visual function in patients with RP.TRIAL REGISTRATIONNCT03063021.FUNDINGMr. and Mrs. Robert Wallace, Mr. and Mrs. Jonathan Wallace, Rami and Eitan Armon, Marc Sumerlin, Cassandra Hanley, and Nacuity Pharmaceuticals, Inc.

Purpose: To characterize the epidemiology and outcomes of hyphema. Methods: Retrospective case series. Medical records from patients with traumatic and spontaneous hyphema seen at the Wilmer Eye Institute, Johns Hopkins, from 2011 through 2017 were evaluated. Aetiology, demographics, clinical characteristics, complications, management and outcomes were ascertained. Multivariable logistic regression was used to identify factors associated with elevated intraocular pressure (IOP), rebleeding and poor outcome (final visual acuity ≤ 20/40) in traumatic hyphema. A safe frequency of follow‐up was retrospectively determined. Results: Traumatic hyphema (n = 152) was more common in males (78%) and adults (55%), with sports/recreational activities being the most frequent cause (40%). Elevated IOP was the most common complication (39%). Rebleeding occurred in seven patients (5%) and was more likely with a higher IOP on presentation (OR:1.1; p = 0.004). Thirty‐seven patients (24%) had a poor outcome, mostly due to traumatic sequelae such as cataract (32%) or posterior segment involvement (30%). A poor outcome was more likely with worse presenting visual acuity (OR: 9.1; p = 0.001), rebleeding (OR: 37.5; p = 0.035) and age > 60 years (OR: 16.0; p = 0.041). Spontaneous hyphema (n = 28) did not have a gender predominance and was more common in adults > 60 years (71%). The most common cause was iris neovascularization (61%). Complications and visual outcomes were worse compared with traumatic hyphema. Conclusions: Traumatic hyphema continues to be common in young males engaging in sports, necessitating increased awareness for preventive eyewear. Older age and rebleeding can lead to poor outcomes. Elevated IOP at presentation predisposes to rebleeding and warrants frequent follow‐up. Otherwise, routine follow‐up at days 1, 3, 5, 7 and 14 is sufficient for uncomplicated cases. [ABSTRACT FROM AUTHOR]

Purpose: To report the clinical characteristics, complications, and outcomes of hyphema in patients with sickle cell trait (SCT). Design: Retrospective case series. Methods: Medical records of SCT patients (confirmed by hemoglobin electrophoresis) presenting with hyphema at the Wilmer Eye Institute over 10 years (January 2008 through December 2017) were reviewed in detail. Data were collected regarding etiology, demographics, clinical course and management. Main outcome measures included intraocular pressure (IOP) and visual acuity (VA). Results: A total of 14 black patients (males: 57%; median age: 20 years) were included in the study with a median follow-up of 4 months (range: 1 week to 6 years). Ten patients had traumatic hyphema with a mean presenting VA of approximately 1.10 logMAR (Snellen equivalent=20/250) and a mean presenting IOP of 40 mmHg. Nine of them required medical intervention to lower IOP, and six of them ultimately required surgical intervention, primarily in the form of anterior chamber paracentesis and/or washout. The mean time to achieve IOP control was 3 days, and the mean final VA was approximately 0.10 logMAR (Snellen Equivalent=20/25). Four patients had non-traumatic hyphema with a mean presenting VA of approximately 1.8 logMAR (Snellen equivalent=20/1260) and a mean presenting IOP of 31 mmHg. Three of them required medical intervention to lower IOP, and two of them ultimately required surgical intervention. Overall, seven patients underwent anterior chamber washout, six needed an anterior chamber paracentesis to lower the IOP, two required tube shunt placement procedures and one patient underwent a trabeculectomy. The mean time to achieve IOP control was 5 days, and the mean final VA was approximately 0.80 logMAR (Snellen equivalent=20/125). Conclusion: Patients with SCT are likely to develop elevated IOP in the setting of hyphema, with a majority requiring one or more surgical interventions to achieve IOP control. [ABSTRACT FROM AUTHOR]