Purpose of reviewThe current article will update and review the clinical and radiological manifestations and management of rhino-orbital mucormycosis (ROM).Recent findingsThere has been an increase in cases of ROM worldwide, especially in India. Immunosuppression (especially diabetes mellitus) is a known predisposing risk factor for ROM. Delayed diagnosis and treatment of ROM can be vision or life-threatening. This article reviews the clinical and radiologic features, treatment, and prognosis of ROM with special emphasis on new and emerging therapies.SummaryROM is an angioinvasive fungal infection that affects the sinuses and orbits and may present to ophthalmologists. Clinicians should have a high index of suspicion for ROM, especially in patients with poorly controlled diabetes mellitus or other immunosuppression. Corticosteroid treatment (including the recent COVID-19 pandemic) may be a predisposing risk factor for ROM.
Purpose of reviewTo review recent therapeutic advances in Leber hereditary optic neuropathy (LHON).Recent findingsIdebenone, a synthetic analog of ubiquinone (Coenzyme Q10) is an antioxidant and component of the mitochondrial electron transport chain. Since the initial approval of the drug in 2015 in Europe, recent trials have evaluated its role as prolonged treatment in LHON. Gene therapy has recently emerged as a promising alternative for the treatment of LHON. Among several investigations, RESCUE and REVERSE are two phase 3 clinical trials of gene therapy in patients with LHON in early stages. Results in these trials have shown a bilateral visual acuity improvement with unilateral intravitreal injections at 96 weeks and sustained visual improvement after 3 years of treatment. The most recent REFLECT phase 3 clinical trial in LHON has shown significant improvement of vision after bilateral intravitreal injections compared with the group that received unilateral injections.SummaryHistorically, LHON has been considered an untreatable disease, but recent developments show that new pharmacological and gene therapy approaches may lead to visual recovery. Further studies are needed to support these data.
Leber hereditary optic neuropathy (LHON) is the most common primary mitochondrial DNA disorder, presenting typically as a sequential, painless, subacute, optic neuropathy in young males. Despite the very limited therapeutic options in LHON, recent developments involving novel pharmacological agents and emerging gene therapy interventions have shown promising results for improved visual outcomes. A synthetic analogue of coenzyme Q (idebenone) is the most common medical treatment in LHON. In a multicentre, double-blind randomized, placebo-controlled clinical trial (Rescue of Hereditary Optic Disease Outpatient Study [RHODOS]), a dose of 900 mg/day of idebenone for 24 weeks was found to be well tolerated and safe. In a follow-up study (RHODOS-OFU), the visual acuity of 70% of patients enrolled in RHODOS was reassessed 30 months after discontinuation of idebenone. Results from this study suggested that visual acuity continued to improve even after discontinuation of the drug. Gene therapy has recently emerged as a potential treatment for LHON. RESCUE and REVERSE were two phase III clinical trials of viral-mediated gene therapy using lenadogene nolparvovec intravitreal injections in patients with early-stage LHON. Results in these trials have shown long-term safety and bilateral visual acuity improvement after unilateral intravitreal injections at 96 weeks, and sustained visual improvement after 3 years of treatment. The most recent phase III clinical trial in LHON (REFLECT) has shown significant improvement of vision after bilateral intravitreal injections of lenadogene nolparvovec compared with unilateral injections. These promising results suggest that, in the near future, LHON might become the first mitochondrial disorder to benefit from gene therapy.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.