Li Y P, Yi Y Z, Zheng H L
Yan Ke Xue Bao. 1989 Dec;5(3-4):122-6, 78.
Keratoplasty specimens from 19 patients with macular corneal dystrophy (MCD), 11 patients with lattice corneal dystrophy (LCD) and 2 patients with granular corneal dystrophy (GCD) were examined by combinations of histochemistry, electron microscopy and electron--histochemistry. Electron histochemistry disclosed that the deposits of MCD have sulfate chondroitin and another hyaluronidase--resistant glycoaminoglycan and that the deposits of LCD have a little sulfate chondroitin. The authors suggest: (1) the possible pathologic mechanism of MCD is that the keratocytes and endothelial cells synthesize abnormal fibrillogranular material which consists of glycoaminoglycan, glycoprotein and lipid; (2) LCD is a primary localized corneal amyloidosis in which the amyloid deposits may result from corneal epithelial cells and keratocytes with a little sulfate chondroitin; (3) the deposits synthesized by corneal epithelial cells and keratocytes in GCD may result from a genetic defect in processing or synthesizing proteins.
对19例黄斑角膜营养不良(MCD)患者、11例格子状角膜营养不良(LCD)患者和2例颗粒状角膜营养不良(GCD)患者的角膜移植标本进行了组织化学、电子显微镜和电子组织化学联合检查。电子组织化学显示,MCD的沉积物含有硫酸软骨素和另一种抗透明质酸酶的糖胺聚糖,而LCD的沉积物含有少量硫酸软骨素。作者提出:(1)MCD可能的病理机制是角膜细胞和内皮细胞合成了由糖胺聚糖、糖蛋白和脂质组成的异常纤维颗粒物质;(2)LCD是一种原发性局限性角膜淀粉样变性,其中淀粉样沉积物可能由角膜上皮细胞和角膜细胞产生,含有少量硫酸软骨素;(3)GCD中角膜上皮细胞和角膜细胞合成的沉积物可能是由于蛋白质加工或合成中的基因缺陷所致。