Meisler D M, Tabbara K F, Wood I S, Alvarado J A, Biswell R
Ophthalmology. 1985 Feb;92(2):217-22. doi: 10.1016/s0161-6420(85)34049-6.
A family is reported in which two brothers have a bilateral, symmetrical corneal disorder characterized by small yellowish to amber-colored globules arranged in a band-shaped configuration in the central superficial layers of the cornea. The globules have a similar clinical appearance to those found in Bietti's "band-shaped nodular dystrophy," a keratopathy that is nonfamilial, has an onset later in life, and initially involves the peripheral cornea. Light and electron microscopic studies of corneal tissue obtained from the proband showed that the globular deposits autofluoresce, that they stain black with Verhoeff's elastic stain, and that they correspond to an electron-dense material found within corneal epithelial cells and keratocytes and within the extracellular tissues of the anterior cornea. A third, younger brother appears to have the early corneal signs of the condition. Unlike the condition described by Bietti, the keratopathy we report is unique in its familial nature, early onset in life, and initial, marked central corneal involvement.
据报道，有一个家族中，两兄弟患有双侧对称性角膜疾病，其特征为在角膜中央表层有呈带状排列的小黄至琥珀色小球。这些小球的临床外观与在比耶蒂“带状结节性营养不良”中发现的小球相似，后者是一种非家族性角膜病，发病较晚，最初累及周边角膜。对先证者角膜组织进行的光镜和电镜研究表明，球状沉积物自发荧光，用韦尔霍夫弹性染色染成黑色，且它们对应于在角膜上皮细胞、角膜细胞以及角膜前部细胞外组织中发现的电子致密物质。第三个弟弟，即较年轻的弟弟，似乎有该病症的早期角膜体征。与比耶蒂所描述的病症不同，我们所报告的角膜病在家族性、发病早以及最初明显累及角膜中央方面具有独特性。
