| Corneal clouding in alport syndrome. | |
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MedLine Citation:
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PMID: 21045658 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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PURPOSE: : Alport syndrome is a hereditary basement membrane disease that typically involves the kidney, the cochlea, and the eyes. Characteristic ocular problems include posterior polymorphous corneal dystrophy, lenticonus, and dot-and-fleck retinopathy. METHODS: : A 48-year-old male patient with Alport syndrome presented with corneal and retinal changes. In 2003, he was diagnosed with posterior polymorphous corneal dystrophy and received a corneal transplant in his left eye in 2007 because of progressive deterioration in visual acuity. At this time, a lamellar macular hole was diagnosed in his right eye. The removed corneal button was examined by light and electron microscopy and by immunohistochemistry. RESULTS: : Histology revealed not only endothelial changes but also a marked irregular thickening of the epithelial basement membrane and of Bowman layer. Alcian blue staining demonstrated an accumulation of mucopolysaccharides in the Bowman layer. CONCLUSIONS: : The presented changes underline the great variation of ocular disorders related to Alport syndrome. To our knowledge, this is one of the first reports describing histologic corneal findings in Alport syndrome. Only a few cases with accumulation of mucopolysaccharides in the Bowman layer have been described previously, none of them being associated with Alport syndrome. Besides, anterior corneal alterations and corneal clouding seem to be uncommon in patients suffering from Alport syndrome. |
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Authors:
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Martina C Herwig; Nicole Eter; Frank G Holz; Karin U Loeffler |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Cornea Volume: 30 ISSN: 1536-4798 ISO Abbreviation: Cornea Publication Date: 2011 Mar |
Date Detail:
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Created Date: 2011-02-10 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8216186 Medline TA: Cornea Country: United States |
Other Details:
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Languages: eng Pagination: 367-70 Citation Subset: IM |
Affiliation:
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From the *Department of Ophthalmology and †Division of Ophthalmic Pathology, University of Bonn, Bonn, Germany. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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