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对12例正常角膜、12例斑块状角膜营养不良和1例全身性糖胺聚糖贮积症角膜进行形态学和胶性铁超微组化研究。结果表明正常角膜上皮下基底膜和前弹力层有硫酸软骨素,实质层胶原纤维间有硫酸软骨素和硫酸角质素,内皮细胞表面有透明质酸,各种细胞膜上有硫酸肝素存在。斑块状角膜营养不良实质层胶原纤维间硫酸软骨素明显增多,硫酸角质素缺乏,角膜实质细胞和内皮细胞胞质内合成了大量的细纤维状物质和异常的糖胺聚糖。全身性糖胺聚糖贮积症为硫酸肝素在细胞内的蓄积,病变细胞膜上硫酸肝素缺乏。作者认为角膜组织中一定量的糖胺聚糖维持了角膜透明,其分布部位、性质和数量变化均可导致角膜混浊。
Twelve patients with normal cornea, 12 patients with plaque-like corneal dystrophy and 1 patient with systemic glycosaminoglycanosis were examined by morphological and colloidal iron ultra-microhistochemistry. The results showed that there are chondroitin sulfate in the basement membrane and the anterior elastic layer in the normal cornea, chondroitin sulfate and keratan sulfate in the parenchymal collagen fibers, hyaluronic acid on the surface of endothelial cells, and heparin sulfate in various cell membranes. Plaque-like corneal dystrophy parenchymal collagen fibers between the significantly increased chondroitin sulfate, keratin sulfate deficiency, corneal parenchymal cells and endothelial cells in the cytoplasm synthesis of a large number of fine fibrous substances and abnormal glycosaminoglycans. Systemic glycosaminoglycanosis is the accumulation of heparin sulfate in the cells, the lack of heparin sulfate on the diseased cell membrane. The authors believe that a certain amount of glycosaminoglycans in corneal tissue to maintain the cornea transparent, the distribution of parts, nature and quantity changes can lead to corneal opacity.