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• W2149063958 abstract "I would like to inform the readership of the recently revised classification of the corneal dystrophies published in the journal Cornea.1Weiss J.S. Møller H.U. Lisch W. et al.The IC3D Classification of the Corneal Dystrophies.Cornea. 2008; 8: 1-42Google Scholar A version is also available online at www.corneasociety.org. (Accessed on 12/11/2008). In 1890, Arthur Groenouw2Groenouw A. Knötchenförmige Hornhauttrübungen “Noduli Corneae.”.Arch Augenheilkd. 1890; 21: 281-289Google Scholar published the first paper on corneal dystrophies describing granular and macular corneal dystrophy. Subsequently, the term was used to refer to a group of inherited corneal diseases typically bilateral, symmetric, slowly progressive, and without relation to systemic disease or environmental factors.3Waring 3rd, G.O. Rodrigues M.M. Laibson P.R. Corneal dystrophies. I. Dystrophies of the epithelium, Bowman′s layer and stroma.Surv Ophthalmol. 1978; 23: 71-122Abstract Full Text PDF PubMed Scopus (151) Google Scholar However, every ophthalmology resident has been taught exceptions to each aspect of this definition. Posterior polymorphous dystrophy may be unilateral or asymmetric. Epithelial map dot corneal dystrophy may have no hereditary tendency and alterations of levels of antigenic serum keratin sulfate may accompany the corneal changes of macular dystrophy. Some names for individual corneal dystrophies are misleading. Many ophthalmologists are reluctant to make a diagnosis of the entity previously called Schnyder crystalline dystrophy, if there is no evidence of corneal crystals. In fact, only 50% of patients with this disease demonstrate corneal crystalline deposits.4Weiss J.S. Visual morbidity in 34 families with Schnyder's crystalline corneal dystrophy.Trans Am Ophthamol Soc. 2007; 105: 616-648PubMed Google Scholar Genotypic analysis has demonstrated further inaccuracies in the nomenclature with different genes that can cause a single dystrophy phenotype (KRT3 and KRT 12 are associated with Meesmann dystrophy), while one single gene (TGFBI) can result in different allelic dystrophy phenotypes (Reis Bücklers, Thiel-Behnke, granular type 1, granular type 2, lattice type 1). While newer genetic information has enhanced our understanding of the dystrophies, it has also made our phenotypic classification system archaic. The International Committee on the Classification of Corneal Dystrophies was created in 2005 to meet the challenge of revision of the corneal dystrophy nomenclature. An international panel of world experts possessing firsthand experience with the clinical, genetic, and histopathologic findings of all the corneal dystrophies performed a critical evaluation of the literature to remove inaccurate information and distil the facts. The following scheme was developed. A template summarizing genetic, clinical, and pathologic information was prepared for each dystrophy. The anatomic classification continues to organize dystrophies according to the corneal layer chiefly affected. Each dystrophy has been assigned a category from 1 to 4 to indicate the level of evidence supporting the existence of a given dystrophy. These categories are:Category 1. A well-defined corneal dystrophy with known gene.Category 2. A well-defined corneal dystrophy with known gene locus but no specific gene identified to date.Category 3. A well-defined corneal dystrophy with no known gene or gene locus.Category 4. A poorly defined corneal dystrophy with no known gene or gene locus. The category assigned to a specific corneal dystrophy can be expected to change over time as knowledge progressively advances. Eventually, all valid corneal dystrophies should attain the classification of category 1 while some category 4 dystrophies may be reclassified as other known dystrophies or eliminated entirely. The nomenclature can be updated over time as new information becomes available. The nomenclature change has been supported by the Cornea Society and will be included in the upcoming revision of the American Academy of Ophthalmology Basic and Clinical Course Series on Cornea and External Eye Diseases, Section 8." @default.
• W2149063958 created "2016-06-24" @default.
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• W2149063958 date "2009-05-01" @default.
• W2149063958 modified "2023-09-26" @default.
• W2149063958 title "Corneal Dystrophy Classification" @default.
• W2149063958 cites W2000329146 @default.
• W2149063958 doi "https://doi.org/10.1016/j.ophtha.2009.01.047" @default.
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