Central Cloudy Corneal Dystrophy of François

Bramsen, Thorkild; Ehlers, Niels; Baggesen, Laust H.

doi:10.1111/j.1755-3768.1976.tb00435.x

Cited by 19 publications

(6 citation statements)

References 9 publications

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“…The CCT was normal in granular dystrophy of Groenouw (type I), in lattice dystrophy (Haab-Dimmer), in crystalline dystrophy of Schnyder (the cases reported by Ehlers & Matthiessen 1973) and in central cloudy dystrophy of Fransois (the cases reported by Bramsen et al 1976). Reduced thickness was found in macular dystrophy of Groenouw (type 11) (t-test, P < 0.001 when compared with the above mentioned value for young men).…”

Section: Hereditary Dystrophiesmentioning

confidence: 88%

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Central Thickness in Corneal Disorders

Ehlers

Bramsen

1978

Acta Ophthalmologica

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In the single individual the central corneal thickness (CCT) shows only small variations. Therefore CCT has been studied in a number of corneal diseases in order to investigate if this dimension might contribute to the diagnosis or to the understanding of the pathogenesis. Normal CCT was found in hereditary dystrophies with the exception of the macular dystrophy of Groenouw (type II), which showed a significantly reduced thickness. Reduced CCT was found in chronic degenerations of leutic, tuberculous or indefinite nature. Marginal degenerations of Fuchs-Terrien type also showed reduced CCT. The possible role of abiotrophic processes in this corneal thinning is dicussed. Endothelial dysfunction is indicated by increased CCT. This occurs in bullous keratopathy and in many acute disorders. Vascularisation of the cornea does not preclude the occurrence of normal or even reduced thickness.

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Section: Hereditary Dystrophiesmentioning

confidence: 88%

“…T h e frequency distribution does not differ from the normal. Bramsen et al 1976). Reduced thickness was found in macular dystrophy of Groenouw (type 11) (t-test, P < 0.001 when compared with the above mentioned value for young men).…”

mentioning

confidence: 78%

Central Thickness in Corneal Disorders

Ehlers

Bramsen

1978

Acta Ophthalmologica

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“…There are only a few publications describing an entire family with CCDF. [16][17][18] Hence, in descriptions of CCDF patients without a detailed family history, it is impossible to exclude the diagnostic possibility of posterior crocodile shagreen degeneration. 19 Fuchs endothelial corneal dystrophy (FECD) is a very common corneal dystrophy and has a familial basis.…”

Section: Dystrophy Versus Degeneration and Other Questionsmentioning

confidence: 99%

IC3D Classification of Corneal Dystrophies—Edition 2

Weiss

Møller

Aldave

et al. 2015

Cornea

448

511

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“…Although the original publication on central cloudy dystrophy of François28 described a hereditary corneal opacification, there have been only a few other publications that have described an entire family with this disease 29,30. Both articles were written before the advent of genotyping so no genetic information is available.…”

Section: Does Every Single Dystrophy Actually Exist?mentioning

confidence: 99%

The IC3D Classification of the Corneal Dystrophies

Weiss

Møller

Lisch

et al. 2008

Cornea

246

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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.

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Central Cloudy Corneal Dystrophy of François

Cited by 19 publications

References 9 publications

Central Thickness in Corneal Disorders

Central Thickness in Corneal Disorders

IC3D Classification of Corneal Dystrophies—Edition 2

The IC3D Classification of the Corneal Dystrophies

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