Polack F M, Bourne W M, Forstot S L, Yamaguchi T
Am J Ophthalmol. 1980 Apr;89(4):575-84. doi: 10.1016/0002-9394(80)90070-7.
An 8 1/2-year-old girl had posterior polymorphous dystrophy that required keratoplasty. Similar lesions were found in the patient's mother, thus establishing the genetic trend of the disease. Scanning electron microscopy studies showed areas of endothelial cell degeneration and multiple depressions or pits. These seemed the most advanced and characteristic lesions of posterior polymorphous dystrophy. Transmission electron microscopy studies in areas adjacent to the pits showed an abnormally developed Descemet's membrane covered by two or more layers of elongated endothelial-like cells. Cells over Descemet's membrane seemed to deposit some Descemet's membrane material and both fine and coarse collagen fibrils. Cells had prominent mitochondria, normal endoplasmic reticulum, and desmosomal attachments. Cytoplasmic fibrils and microvilli were usually found in cells lining the anterior chamber. Specular microscopy and scanning electron microscopy recorded the image of only the innermost cells and these appeared as endothelium.
一名8岁半的女孩患有后极性多形性营养不良,需要进行角膜移植。在患者母亲身上发现了类似病变,从而确定了该疾病的遗传倾向。扫描电子显微镜研究显示内皮细胞变性区域以及多个凹陷或凹坑。这些似乎是后极性多形性营养不良最严重且最具特征性的病变。在凹坑附近区域进行的透射电子显微镜研究显示,后弹力层发育异常,被两层或更多层伸长的内皮样细胞覆盖。后弹力层上的细胞似乎沉积了一些后弹力层物质以及细胶原纤维和粗胶原纤维。细胞有突出的线粒体、正常的内质网和桥粒连接。在前房内衬细胞中通常可发现细胞质纤维和微绒毛。镜面显微镜和扫描电子显微镜仅记录了最内层细胞的图像,这些细胞看起来像内皮细胞。