Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University College of Medicine, Seoul, Republic of Korea.
Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University College of Medicine, Seoul, Republic of Korea.
Exp Eye Res. 2014 Apr;121:58-65. doi: 10.1016/j.exer.2014.02.003. Epub 2014 Feb 15.
Signature ophthalmic characteristics of Wilson's disease (WD) are regarded as diagnostically important manifestations of the disease. Previous studies have proved the common occurrence of copper accumulation in the liver of patients with WD. However, in the case of sunflower cataracts, one of the rare diagnostic signs of WD, no study has demonstrated copper accumulation in the lens capsules of sunflower cataracts in WD patients. To investigate the nanostructure and elemental composition of sunflower cataracts in WD, transmission electron microscopy (TEM) was done on the capsulorhexised anterior lens capsule of sunflower cataracts in WD in order to evaluate anatomical variation and elemental changes. We utilized energy dispersive X-ray spectroscopy (EDS) to investigate the elemental composition of the lens capsule using both point and mapping spectroscopy. Quantitative analysis was performed for relative comparison of the elements. TEM showed the presence of granular deposits of varying size (20-350 nm), appearing mainly in the posterior one third of the anterior capsule. The deposits appeared in linear patterns with scattered dots. There were no electron-dense particles in the epithelial cell layer of the lens. Copper and sulfur peaks were consistently revealed in electron-dense granular deposits. In contrast, copper and sulfur peaks were absent in other tissues, including granule-free lens capsules and epithelial tissue. Most copper was exclusively located in clusters of electron-dense particles, and the copper distribution overlapped with sulfur on mapping spectroscopy. Quantitative analysis presented inconsistent ratios of copper to sulfur in each electron-dense granule. The mean ratio of copper to sulfur was about 3.25 (with a range of 2.39-3.78). This is the first elemental analysis of single electron particles in sunflower cataracts using EDS in the ophthalmic area. Sunflower cataracts with WD are assumed to be the result of accumulation of heterogeneous compounds composed of several materials, including copper, sulfur, and/or copper-binding proteins. Linear patterns of copper and sulfur deposition were detected in various sizes and composition ratios with these elements in cases of WD.
威尔逊病（WD）的眼部特征被认为是该疾病具有诊断意义的表现之一。既往研究已经证实 WD 患者肝脏存在铜蓄积。然而，在 WD 的一种罕见诊断标志——向日葵白内障中，尚无研究表明 WD 患者晶状体囊中的铜蓄积。为了研究 WD 向日葵白内障的纳米结构和元素组成，我们对 WD 向日葵白内障患者已被囊膜化的前晶状体囊进行了透射电子显微镜（TEM）检查，以评估解剖学变化和元素变化。我们利用能量色散 X 射线光谱（EDS）通过点和映射光谱对晶状体囊的元素组成进行了研究。我们对元素进行了定量分析，以进行相对比较。TEM 显示，大小不一（20-350nm）的颗粒状沉积物的存在，主要出现在前囊的后三分之一。沉积物呈线性模式，散布着点状。晶状体上皮细胞层中没有电子致密颗粒。电子致密颗粒中始终存在铜和硫峰。相比之下，其他组织中（包括无颗粒晶状体囊和上皮组织）不存在铜和硫峰。大多数铜仅位于电子致密颗粒簇中，并且在映射光谱上，铜的分布与硫重叠。定量分析显示每个电子致密颗粒中铜与硫的比值不一致。电子致密颗粒中铜与硫的平均比值约为 3.25（范围为 2.39-3.78）。这是首次在眼科领域使用 EDS 对单个电子颗粒进行的向日葵白内障元素分析。WD 引起的向日葵白内障被认为是由包括铜、硫和/或铜结合蛋白在内的几种物质组成的异质化合物的积累所致。WD 病例中检测到这些元素的不同大小和组成比的线性铜和硫沉积模式。
