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Title: Posterior Polymorphous Dystrophy and Alport Syndrome
Authors: Chaiwat Teekhasaenee
Sumalee Nimmanit
Sorot Wijtthiphan
Kriengsak Vareesangthip
Tawee Laohapand
Prida Malasitr
Robert Ritch
Faculty of Medicine, Ramathibodi Hospital, Mahidol University
Mahidol University
New York Eye and Ear Infirmary
Keywords: Medicine
Issue Date: 1-Jan-1991
Citation: Ophthalmology. Vol.98, No.8 (1991), 1207-1215
Abstract: Abstract: Seventeen Thai patients from nine families with Alport syndrome underwent complete ocular examination and specular microscopy. Fourteen (82.3%) patients had ocular changes. Eleven (64.7%) had endothelial vesicles compatible with posterior polymorphous dystrophy. Four of these also had subepithelial opacities, a previously undescribed phenomenon. Other ocular changes included lenticonus and macular and midperipheral retinal flecks. A second group of 18 consecutive patients from 14 families with posterior polymorphous dystrophy detected during routine ocular examination underwent renal evaluation. Five had hematuria, four of whom had sensorineural hearing loss. Two of the four patients also had characteristic renal biopsy findings. Another had sensorineural hearing loss without hematuria, and renal biopsy showed a thin glomerular basement membrane. Posterior polymorphous dystrophy is a common but frequently overlooked finding in Alport syndrome. The frequent association of these two hereditary conditions suggests a common defect in basement membrane formation. Patients with posterior polymorphous dystrophy should be examined for renal abnormalities and hearing loss. © 1991, American Academy of Ophthalmology, Inc. All rights reserved.
ISSN: 01616420
Appears in Collections:Scopus 1991-2000

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