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伴有角膜淀粉样变性的气候性滴状角膜病变

Climatic droplet keratopathy with corneal amyloidosis.

作者信息

Matta C S, Tabbara K F, Cameron J A, Hidayat A A, al-Rajhi A A

机构信息

King Saud University, College of Medicine, Riyadh.

出版信息

Ophthalmology. 1991 Feb;98(2):192-5. doi: 10.1016/s0161-6420(91)32317-0.

Abstract

Climatic droplet keratopathy (CDK) is a degenerative disease of the cornea, highly prevalent in Saudi Arabia. It is characterized by aggregates of small, golden-yellow globules that accumulate in the subepithelial layers, Bowman's layer, and the superficial stroma of the cornea. The authors report on 22 patients (40 eyes) with CDK and lattice lines in the cornea. The condition occurred late in life (mean age, 67 years). It was bilateral in 82% of patients, with no family history of lattice dystrophy or systemic amyloidosis. These lines were found in all layers of the stroma. Results of histopathologic and electron microscopic examination of the lattice lines confirmed the presence of amyloid. Results of immunohistochemical examination showed evidence of deposition of AP protein. The cause of these deposits in the cornea is not known, but the authors speculate that environmental factors causing CDK also may play a role in the deposition of protein AP in the cornea. The contribution of trachoma to the degenerative changes in the cornea and secondary amyloidosis could not be ruled out, as all patients had inactive trachoma. Climatic droplet keratopathy with corneal amyloidosis, therefore, represents a form of "lattice degeneration" of the cornea.

摘要

气候性滴状角膜病变(CDK)是一种角膜退行性疾病,在沙特阿拉伯极为常见。其特征是角膜上皮下、Bowman层及角膜浅层基质中出现小的金黄色球状物聚集。作者报告了22例(40只眼)患有CDK且角膜有格子样线条的患者。这种情况在晚年出现(平均年龄67岁)。82%的患者为双侧患病,无格子状营养不良或系统性淀粉样变性家族史。这些线条存在于基质的所有层中。对格子样线条进行组织病理学和电子显微镜检查的结果证实了淀粉样物质的存在。免疫组织化学检查结果显示有AP蛋白沉积的证据。角膜中这些沉积物的病因尚不清楚,但作者推测导致CDK的环境因素可能也在角膜中AP蛋白的沉积中起作用。由于所有患者均患有静止期沙眼,因此不能排除沙眼对角膜退行性变和继发性淀粉样变性的影响。因此,伴有角膜淀粉样变性的气候性滴状角膜病变代表了角膜“格子样变性”的一种形式。

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