TY - JOUR
T1 - La Clasificación IC3D de las distrofias corneales
AU - Weiss, Jayne S.
AU - Møller, H. U.
AU - Lisch, Walter
AU - Kinoshita, Shigeru
AU - Aldave, Anthony J.
AU - Belin, Michael W.
AU - Kivelä, Tero
AU - Busin, Massimo
AU - Munier, Francis L.
AU - Seitz, Berthold
AU - Sutphin, John
AU - Bredrup, Cecilie
AU - Mannis, Mark J
AU - Rapuano, Christopher J.
AU - Van Rij, Gabriel
AU - Kim, Eung Kweon
AU - Klintworth, Gordon K.
PY - 2008/12
Y1 - 2008/12
N2 - 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 welldefined 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.
AB - 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 welldefined 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.
KW - Corneal dystrophy
KW - Corneal histopathology
KW - Epithelial basement membrane dystrophy
KW - Epithelial recurrent erosion dystrophy
KW - Eponym
KW - Gene
KW - Genetic corneal disease
KW - Inherited corneal disease
KW - Key reference
KW - Mutation
KW - Subepithelial mucinous corneal dystrophy
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UR - http://www.scopus.com/inward/citedby.url?scp=65349167054&partnerID=8YFLogxK
U2 - 10.1097/ICO.0b013e31817780fb
DO - 10.1097/ICO.0b013e31817780fb
M3 - Article
C2 - 19337156
AN - SCOPUS:65349167054
VL - 27
JO - Cornea
JF - Cornea
SN - 0277-3740
IS - SUPPL. 2
ER -