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Light and Electron Microscopic Studies of the Variant Type of Reis-Bucklers' Corneal Dystrophy-Case Report
雷斯－巴克角膜失養症變異型之顯微研究－病例報告
摘要
雷斯－巴克(Reis-Bücklers')角膜失養症是一種影響到角膜表皮的基質細胞，Bowman氏層，基質前層的一種角膜失養症。而其變異型Thiel-Behnke's失養症與其有共同的一些特色：反覆性的表皮破損及晚期時視力的喪失。本研究側重於一有家族病史的四十六歲男子經診斷為Thiel-Behnke's失養症的角膜前部顯微結構。構造中發現多處表皮基膜的缺乏、Bowman氏層的破壞、及位於表皮基膜與Bowman氏層間典型的”peculiar-curly”纖維的沉積。
關鍵字
無資料並列摘要
Reis-Bücklers' corneal dystrophy is a type of anterior corneal dystrophy involving the Bowman's membrane, anteior stroma, and basal cells of the corneal epithelium. Despite of the similar clinical characteristics: recurrent epithelial erosion, and progressive visual loss in a later stage; the variant, Thiel-Behnke's dystrophy, had the typical electron microscopic findings. The literature presents the ultrastructural characteristics of epithelium, Bowman's layer, and anterior stroma from the corneal tissue of a 46 years old adult with the diagnosis of Thiel-Behnke's dystrophy in an affected family of three generations. The tissue reveals absence of the epithelial basement membrane in many areas, destruction of Bowman's layer, and areas of patchy deposition of characteristic ”peculiar curly” filaments between a distorted epithelial basal lam ma and Bowman's layer.
延伸閱讀
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