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赖斯-比克勒营养不良症。

Reis-Bückler's dystrophy.

作者信息

Pouliquen Y, Giraud J P, Savoldelli M

出版信息

Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1978 Nov 8;208(1-3):25-31. doi: 10.1007/BF00406979.

Abstract

A case of Reis-Bückler's dystrophy in a 36-year-old man is reported. Its clinical aspect is compared with its histological and ultrastructural features. The slight reticular opacities situated superficially in the central part of the cornea, immediately beneath the epithelium, correspond to dark, irregular deposits. These replaced the basal membrane and Bowman's membrane and are composed of granular material, glycogen granules, and short fibers. These short, curved, osmiophilic fibers whose diameter is approximately 130 A are also located inside the anterior stromal lamellae. These deposits seem to be the characteristic feature of this particular and rare dystrophy, stated as by Hogan. Their nature and origin are discussed.

摘要

报道了一名36岁男性患 Reis-Bückler 营养不良症的病例。将其临床症状与其组织学和超微结构特征进行了比较。角膜中央部分上皮下方浅表处的轻微网状混浊对应于深色不规则沉积物。这些沉积物取代了基底膜和Bowman膜,由颗粒物质、糖原颗粒和短纤维组成。这些直径约为130埃的短的、弯曲的、嗜锇性纤维也位于前基质板层内。正如 Hogan 所述,这些沉积物似乎是这种特殊罕见营养不良症的特征性表现。讨论了它们的性质和起源。

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