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과립형 각막변성 Granular Corneal Dystrophyq

대한안과학회지 1976년 17권 2호 p.229 ~ 233
박병은, 김경식,
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박병은 (  ) 
연세대학교 의과대학 안과학교실

김경식 (  ) 
연세대학교 의과대학 안과학교실

Abstract


Granular corneal dystrophy is characterized by the presence of milk-white spots in the superficial stroma typically lying underneath Bowman´s membrane in the axial region of the cornea. This corneal dystrophy was first described by Groenouw in 1890. Since then, not only this dystrophy but also many other types of familial corneal dystrophies have been described. In 1938 B>icklersclassified the corneal dystrophy into three types ; granular, lattice and macular corneal dystropy. Granular corneal ´dystrophy was inherited es an autosomal dominant characteristics. It begins in. the first decade of life becoming obious at about the age of puberty. The early leasions aresmall, discrete, grayish-white spots in the superficial stroma of both corneas, and as the condition advances, the lesions are more evident and of various sizes and shapes. This opacities are confined mostly to the axial portion of the cornea. There is no decrease in coreal sensitivity nor any vascularization. Gradually the opacities enlarge, thicken and coalesce into irregular granules hooks, rings and streaks of whitish color and glassy structure. The progress of the disease is usually slow, and moderately good vision often remains in the fourth or fifth decade. The main histopathologic feature is the deposition of a hyalin-like material in the corneal stroma. When the opacification or the irritative episodes become disabling, corneal grafting may be indicated.
The cases reported here are granular dystrophies which involved four daughters of one family.

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