The mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by inborn errors of glycosaminoglycan (GAG) metabolism. These diseases are classified by enzyme deficiency into seven groups: type I, II, III, IV, VI, VII, and IX. GAG accumulation leads to characteristic clinical features. Some ophthalmic findings that are characteristic of MPS diseases include corneal clouding, retinal degeneration, decreased electroretinogram wave amplitude, optic atrophy, papilledema, and glaucoma. Current treatments such as hematopoietic stem cell transplantation and enzyme replacement therapy have increased the life span of many MPS patients and created the need to improve management of ocular symptoms. This article aims to provide a comprehensive review of ocular manifestations and treatment options for the various types of MPS.
Purpose: To evaluate the efficacy and safety of monthly intravitreal injections of ranibizumab in patients with polypoidal choroidal vasculopathy (PCV) and active exudation or hemorrhage. Methods: A prospective, single practice, open label trial of monthly intravitreal ranibizumab (0.5 mg) injections for PCV in 13 eyes of 13 patients who completed the 1-year study. The primary outcome measure was stabilization of vision (loss of <15 ETDRS letters). Secondary outcome measures included incidence of ocular and systemic adverse events, changes in subretinal hemorrhage, central foveal thickness, and polypoidal complexes on indocyanine green angiography at 1 year. Results: No patient lost ≥15 letters in visual acuity at 1 year. Three patients (23%) gained ≥15 letters at 12 months. Subretinal hemorrhage resolved in 9/9 eyes (100%). Macular edema improved in 5/5 eyes (100%). Subretinal fluid completely resolved in 4/9 eyes (44%), decreased in 2/9 eyes (22%), and increased in 3/9 eyes (33%). Polypoidal complexes decreased in 5/13 eyes (38%). Conclusion: Continuous monthly intravitreal ranibizumab decreases leakage and hemorrhage in eyes with exudative and hemorrhagic complications of PCV. Branching vascular networks persisted, and polypoidal complexes decreased in only 5/13 (38%) eyes with continuous antiangiogenic therapy at 1 year.
Purpose: To report a case of reversible corneal endothelial abnormalities after treatment with netarsudil. Observation: A 68-year-old woman presented with the complaint of blurred vision soon after starting treatment with the fixed-dose combination of netarsudil and latanoprost (FC-netarsudil-latanoprost). She had been receiving the fixed-dose combination of dorzolamide and timolol and latanoprost for primary open-angle glaucoma until her ophthalmologist switched latanoprost to FC-netarsudil-latanoprost 2 months before referral to our center. Best-corrected visual acuity was 20/20-1 in the right eye and 20/20-3 in the left eye. The slit-lamp biomicroscopic examination was remarkable for a guttata-like abnormality of the corneal endothelium of both eyes. The intraocular pressure was 10 mm Hg in both eyes. Specular microscopy revealed irregularly shaped corneal endothelial cells with indistinct borders between cells. FC-netarsudil-latanoprost was replaced with latanoprost in the left eye but continued in the right eye. Nine weeks later, best-corrected visual acuity remained 20/20-1 in the right eye but it improved to 20/20 in the left eye. Repeat specular microscopy was unchanged in the right eye and was normal in the left eye. Conclusion and Importance: Topical therapy with netarsudil can result in guttata-like changes of the corneal endothelium and corneal endothelial cell abnormalities that can be detected with specular microscopy. These abnormalities seem to be transient and resolved upon the cessation of netarsudil. Ophthalmologists should consider the possibility of a corneal endothelial abnormality in patients treated with netarsudil who develop blurred vision.
Molecular typing reveals that CipR within the tested population is maintained by strain turnover between resistant genogroups. Despite early recommendation in 2002 to stop ciprofloxacin use in California, CipR in SF increased through 2006. The subsequent decrease in CipR corresponds with the 2007 national recommendation to cease ciprofloxacin treatment of gonorrhea, which suggests that national recommendations are potentially more effective at reducing CipR than regional recommendations in areas with high strain turnover.
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