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Lattice corneal dystrophy : ウィキペディア英語版
Lattice corneal dystrophy

Lattice corneal dystrophy type, also known as Biber-Haab-Dimmer dystrophy, is a rare form of corneal dystrophy. It has no systemic manifestations, unlike the other type of the dystrophy, Lattice corneal dystrophy type II. Lattice corneal dystrophy was first described by Swiss ophthalmologist Hugo Biber in 1890.〔H. Biber: Über einige seltenere Hornhauterkrankungen. Zürich, 1890. Inaugural Dissertation, Zurich, 1890. Cited by O. Haab: Die gittrige Keratitis.〕
Lattice dystrophy gets its name from an accumulation of amyloid deposits, or abnormal protein fibers, throughout the middle and anterior stroma.
== Genetics ==

Lattice corneal dystrophy has two types:
* type I: with no systemic association. It is caused by mutations in TGFBI gene encoding keratoepithelin, which maps to chromosome 5q.
* type II or Finnish type amyloidosis: associated with manifestations of systemic amyloidosis due to accumulation of gelsolin. Associated conditions may include cutis laxa and ataxia.
* type III is also described which has an onset at age 70 to 90 years and is not associated with systemic amyloidosis.〔

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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