Korean J Ophthalmol > Volume 19(1); 2005 > Article
Korean Journal of Ophthalmology 2005;19(1):84-89.
DOI: https://doi.org/10.3341/kjo.2005.19.1.84    Published online March 30, 2005.
Anterior Lens Capsule Abnormalities in Alport Syndrome.
Jae Hyuk Choi, Kyung Sool Na, Seon Hee Bae, Gyoung Hwan Roh
Department of Ophthalmology, College of Medicine, Konyang University, Myung Gok Eye Research Institute, Daejeon, Korea. eyedr00@yahoo.co.kr
Alport syndrome is a hereditary, progressive disease characterized by progressive nephritis, sensorineural deafness, and ocular abnormalities, including anterior lenticonus. The ultrastructure of the lens capsule abnormalities in Alport syndrome is reported. Four anterior lens capsules from 31-year-old patient and 26-year-old patient with lenticonus who were affected by the Alport syndrome were obtained at capsulectomy. And all four anterior lens capsules were examined by transmission electron microscopy. The histopathologic findings showed that the thickness of the anterior lens capsules was decreased (4~13 micrometer) and that there were many vascular dehiscences localized at the inner part of the lens capsule. There were large numbers of capsular dehiscences containing fibrillar materials and vacuoles. The anterior capsules were clearly fragile in this disease, forming the basis for the progressive lenticonus and anterior polar cataract.
Key Words: Alport syndrome;Capsular dehiscence;Lenticonus

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