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Fabry keratopathy: Manifestations and changes over time

British Journal of Ophthalmology Nov 08, 2019

Sivley MD, et al. - Researchers examined the clinical perceptibility for the changes in Fabry keratopathy over time. In addition, they examined variations of Fabry keratopathy, supplemental features, unique presentations and differences between keratopathies of right and left corneas and the same family and genotype. In 10 persons (5 men and 5 women) with classic Fabry disease, they captured Biomicroscopic images of Fabry keratopathy over an 18-month period. Changes in Fabry keratopathy ranged from mild to marked over 18 months. A great variety of whorl patterns were observed. A few keratopathies were amorphous without vortices and many vortices were supplemented with amorphous features. They recommend practitioners to be better able to identify Fabry disease via having a fuller understanding of the variety of presentations of the dynamic, pathognomonic Fabry keratopathy. Spatially unique routes of continuous centripetal renewal of corneal epithelium were observed in each eye, in some cases subsidized by the direct contribution of basal cells streamed from the superior limbus.
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