The IC3D classification of the corneal dystrophies.

Details

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Version: author
Serval ID
serval:BIB_2F40B810736B
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
The IC3D classification of the corneal dystrophies.
Journal
Cornea
Author(s)
Weiss J.S., Møller H.U., Lisch W., Kinoshita S., Aldave A.J., Belin M.W., Kivelä T., Busin M., Munier F.L., Seitz B., Sutphin J., Bredrup C., Mannis M.J., Rapuano C.J., Van Rij G., Kim E.K., Klintworth G.K.
ISSN
1536-4798[electronic]
Publication state
Published
Issued date
2008
Peer-reviewed
Oui
Volume
27 Suppl 2
Pages
S1-83
Language
english
Notes
Publication types: Historical Article ; Journal Article ; Review - Publication Status: ppublish
Abstract
BACKGROUND: The recent availability of genetic analyses has demonstrated the shortcomings of the current phenotypic method of corneal dystrophy classification. Abnormalities in different genes can cause a single phenotype, whereas different defects in a single gene can cause different phenotypes. Some disorders termed corneal dystrophies do not appear to have a genetic basis. PURPOSE: The purpose of this study was to develop a new classification system for corneal dystrophies, integrating up-to-date information on phenotypic description, pathologic examination, and genetic analysis. METHODS: The International Committee for Classification of Corneal Dystrophies (IC3D) was created to devise a current and accurate nomenclature. RESULTS: This anatomic classification continues to organize dystrophies according to the level chiefly affected. Each dystrophy has a template summarizing genetic, clinical, and pathologic information. A category number from 1 through 4 is assigned, reflecting the level of evidence supporting the existence of a given dystrophy. The most defined dystrophies belong to category 1 (a well-defined corneal dystrophy in which a gene has been mapped and identified and specific mutations are known) and the least defined belong to category 4 (a suspected dystrophy where the clinical and genetic evidence is not yet convincing). The nomenclature may be updated over time as new information regarding the dystrophies becomes available. CONCLUSIONS: The IC3D Classification of Corneal Dystrophies is a new classification system that incorporates many aspects of the traditional definitions of corneal dystrophies with new genetic, clinical, and pathologic information. Standardized templates provide key information that includes a level of evidence for there being a corneal dystrophy. The system is user-friendly and upgradeable and can be retrieved on the website www.corneasociety.org/ic3d.
Keywords
Corneal Dystrophies, Hereditary/classification, Corneal Dystrophies, Hereditary/genetics, History, 19th Century, Humans, International Cooperation, Ophthalmology/trends, Phenotype, Terminology as Topic
Pubmed
Web of science
Open Access
Yes
Create date
05/10/2009 15:44
Last modification date
20/08/2019 13:13
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