ArticleTransactions of the American Ophthalmological Society2007
Visual morbidity in thirty-four families with Schnyder crystalline corneal dystrophy (an American Ophthalmological Society thesis).
Article in Transactions of the American Ophthalmological Society, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 51 citations in OpenAlex.
- Allosteric regulation of UBIAD1 trafficking from ER to Golgi revealed by chemical genetic screening.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Case Report of Schnyder Corneal Dystrophy-A Rare Lipid Metabolic Disorder of the Cornea.Life (Basel, Switzerland) · 2025Article
- [The latest IC3D classification of corneal dystrophies-Overview and changes of the 3rd edition].Die Ophthalmologie · 2024Review
- Vitamin K and the Visual System-A Narrative Review.Nutrients · 2023Review
- Management of Stromal Corneal Dystrophies; Review of the Literature with a Focus on Phototherapeutic Keratectomy and Keratoplasty.Vision (Basel, Switzerland) · 2023Review
- Case report: A case of corneal deposits between binocular descemet membrane and corneal endothelial layer after small-incision lenticule extraction (SMILE) followed by HPV vaccine.Frontiers in medicine · 2022Article
- Functional study of SCCD pathogenic geneMolecular medicine reports · 2021Article
- Corneal dystrophies.Nature reviews. Disease primers · 2020Review
- Schnyder corneal dystrophy-associated UBIAD1 is defective in MK-4 synthesis and resists autophagy-mediated degradation.Journal of lipid research · 2020Article
- Multimodal Imaging Features of Schnyder Corneal Dystrophy.Journal of ophthalmology · 2020Article
- Article
- Heredity and in vivo confocal microscopy of punctiform and polychromatic pre-Descemet dystrophy.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2018Article
- A Mouse Model of Schnyder Corneal Dystrophy with the N100S Point Mutation.Scientific reports · 2018Article
- The Hematologic Definition of Monoclonal Gammopathy of Undetermined Significance in Relation to Paraproteinemic Keratopathy (An American Ophthalmological Society Thesis).Transactions of the American Ophthalmological Society · 2016Article
- Bringing Bioactive Compounds into Membranes: The UbiA Superfamily of Intramembrane Aromatic Prenyltransferases.Trends in biochemical sciences · 2016Review
- Identification of the First De Novo UBIAD1 Gene Mutation Associated with Schnyder Corneal Dystrophy.Journal of ophthalmology · 2016Article
- The prenyltransferase UBIAD1 is the target of geranylgeraniol in degradation of HMG CoA reductase.eLife · 2015Article
- Crystalline subtype of pre-descemetic corneal dystrophy.Journal of ophthalmic & vision research · 2014Article
- The UBIAD1 prenyltransferase links menaquinone-4 [corrected] synthesis to cholesterol metabolic enzymes.Human mutation · 2013Article
- Clinical and pathological features of a non-crystalline form of Schnyder corneal dystrophy.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2012Article
Corrections and comments
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Authors and funding
1 author at 1 institution in 1 country.
Funding
Abstract
purposeTo assess the findings, visual morbidity, and surgical intervention in Schnyder crystalline corneal dystrophy (SCCD).
methodsRetrospective case series of 115 affected individuals from 34 SCCD families identified since 1989. Age, uncorrected visual acuity, best-corrected visual acuity (BCVA), corneal findings, and ocular surgery were recorded. Prospective phone, e-mail, or written contact provided updated information. Patients were divided into 3 age categories for statistical analysis: less than 26 years of age, 26 to 39 years of age, and 40 years of age and older.
resultsMean age on initial examination was 38.8 +/- 20.4 (range, 2-81) with follow-up of 55 of 79 (70%) of American patients. While there were no statistical significant correlations between logMAR visual acuity and age (logMAR BCVA =.033 + .002 x age; R =.21), the linear regression showed the trend of worse visual acuity with age. BCVA at > or =40 years was decreased compared to <40 (P < .0001), although mean BCVA was > 20/30 in both groups. Twenty-nine of 115 patients had corneal surgery with 5 phototherapeutic keratectomy (3 patients), and 39 penetrating keratoplasty (PKP) (27 patients). PKP was reported in 20 of 37 (54%) patients > or =50 years and 10 of 13 (77%) of patients > or =70. BCVA 1 year prior to PKP in 15 eyes (9 patients) ranged from 20/25 to 20/400 including 7 eyes with other ocular pathology. BCVA in the remaining 8 eyes was 20/25 to 20/70 with 3 of these 4 patients reporting preoperative glare. Chart and phone survey suggested increasing difficulty with photopic vision with aging.
conclusionAlthough excellent scotopic vision continues until middle age in SCCD, most patients had PKP by the 7th decade. SCCD causes progressive corneal opacification, which may result in glare and disproportionate loss of photopic vision.
Indexed as
Identifiers
18427632PMC2258126W2168293280What OpenQuestion holds
Registered trials
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