title: Elemental analysis of sunflower cataract in Wilson's disease: a study using scanning transmission electron microscopy and energy dispersive spectroscopy.
abstract: 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.
authors.affiliation: Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University College of Medicine, Seoul, Republic of Korea.
authors.forename: Hyo Ju
authors.initials: HJ
authors.lastname: Jang
authors.affiliation: Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University College of Medicine, Seoul, Republic of Korea.
authors.forename: Joon Mo
authors.initials: JM
authors.lastname: Kim
authors.affiliation: Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University College of Medicine, Seoul, Republic of Korea. Electronic address: sashimi0@naver.com.
authors.forename: Chul Young
authors.initials: CY
authors.lastname: Choi
mesh_terms: D000328:Adult; D057866:Anterior Capsule of the Lens / Q000378:metabolism* / Q000648:ultrastructure; D002352:Carrier Proteins / Q000378:metabolism*; D002386:Cataract / Q000175:diagnosis / Q000378:metabolism*; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000378:metabolism*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D017348:Microscopy, Electron, Scanning Transmission; D018918:Phacoemulsification; D013052:Spectrometry, X-Ray Emission; D013457:Sulfur Compounds / Q000378:metabolism*
chemical_list: D002352:Carrier Proteins; D013457:Sulfur Compounds; C023548:copper-binding protein
keywords: Wilson's disease; elemental analysis; energy dispersive X-ray spectroscopy; scanning transmission electron microscopy; sunflower cataract