ArticleAmerican journal of ophthalmology case reports2018
Bull's eye maculopathy and subfoveal deposition in two mucopolysaccharidosis type I patients on long-term enzyme replacement therapy.
Article in American journal of ophthalmology case reports, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 18 citations in OpenAlex.
- Article
- The Role of Visual Electrophysiology in Systemic Hereditary Syndromes.International journal of molecular sciences · 2025Review
- Bilateral Foveal Cysts in Mucopolysaccharidosis Type I (Hurler Syndrome): Response to Acetazolamide With Insights From Multimodal Retinal Imaging and Electrophysiology.Case reports in ophthalmological medicine · 2025Article
- Posterior segment findings in Hunter Syndrome: Case report and review.American journal of ophthalmology case reports · 2024Article
- Clinical and pathological characterization of ophthalmic disease in a canine model of mucopolysaccharidosis type I.Journal of inherited metabolic disease · 2023Article
- Autophagy in the retinal pigment epithelium: a new vision and future challenges.The FEBS journal · 2022Review
- Macular Changes in a Mucopolysaccharidosis Type I Patient with Earlier Systemic Therapies.Case reports in ophthalmological medicine · 2021Article
- Sialidosis type II: Expansion of phenotypic spectrum and identification of a common mutation in seven patients.Molecular genetics and metabolism reports · 2020Article
- Ocular features in mucopolysaccharidosis: diagnosis and treatment.Italian journal of pediatrics · 2018Review
- Bull's eye maculopathy and subfoveal deposition in two mucopolysaccharidosis type I patients on long-term enzyme replacement therapy.American journal of ophthalmology case reports · 2018Article
- Recovery of Vision following Enzyme Replacement Therapy in a Patient with Mucopolysaccharidosis Type II, Hunter Syndrome.Case reports in ophthalmologyArticle
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeTo report retinal findings in two patients with mucopolysaccharidosis type I (MPS I) receiving human recombinant alpha-l-iduronidase (Laronidase) as enzyme replacement therapy. OBSERVATIONS: Patient 1 had visual acuity 20/20 right eye, 20/25 left eye and unremarkable anterior segment and retinal examination. Optical coherence tomography (OCT) scanning demonstrated parafoveal thinning and subfoveal hyperreflectant material. Patient 2 had visual acuity 20/20 both eyes, with dense nuclear cataract both eyes. Retinal examination demonstrated bull's eye maculopathy both eyes. OCT scanning confirmed parafoveal atrophy and demonstrated similar appearing subfoveal hyperreflectant material, more prominent than in case 1. CONCLUSIONS AND IMPORTANCE: These two patients with MPS I receiving Laronidase treatment have developed bull's eye maculopathy changes and subfoveal deposition of hyperreflectant material despite excellent compliance and good tolerance of the standard dose of enzyme therapy for this disorder. Further studies are required to determine the nature of the material, the incidence and the effect of enzyme replacement therapy on these findings in patients with MPS I.
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