PMID: 8594632Jan 1, 1995Paper

Hyperlipofuscinosis and subretinal fibrosis in Stargardt's disease

Retina
J J De Laey, C Verougstraete

Abstract

The fundus aspect of Stargardt's disease is characterized by progressive atrophic macular changes often associated with fundus flavimaculatus flecks. Other fundus changes have been described such as pallor of the optic disc, attenuated retinal vessels, cicatricial chorioretinitis, retinal pigment hyperplasia, subretinal neovascularization, or subretinal fibrosis. Three cases of Stargardt's disease and presumed subretinal hyperlipofuscinosis or subretinal fibrosis are presented. The clinical history, and the ophthalmoscopic and fluoroangiographic characteristics of these three patients are discussed, as well as the clinical course in the two first patients. Two young patients with the ophthalmoscopic and fluoroangiographic aspects of Stargardt's disease in one eye showed the presence of dense orange subretinal material in the posterior pole of the fellow eye. During the follow-up period, the abnormal material regressed partially in one patient and completely in the other, leaving subretinal fibrosis and localized retinal pigment epithelial hyperplasia. The left fundus in a third patient was characterized by an area of subretinal fibrosis in the midperiphery. It is presumed that the abnormal material in the two first patients is ...Continue Reading

Citations

Jan 16, 2008·The Journal of Biological Chemistry·Marcin GolczakKrzysztof Palczewski
Feb 26, 2009·The Journal of Biological Chemistry·Monika Damek-PoprawaKathleen Boesze-Battaglia
Sep 14, 2006·Annual Review of Pharmacology and Toxicology·Gabriel H TravisKrzysztof Palczewski
Jan 24, 2013·Case Reports in Ophthalmology·Settimio RossiFrancesca Simonelli
Mar 21, 2002·Proceedings of the National Academy of Sciences of the United States of America·Silvia C FinnemannEnrique Rodriguez-Boulan
Oct 31, 2014·Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire·Mustapha Lhor, Christian Salesse
Jun 8, 2011·Eye & Contact Lens·Randolph D Glickman
Jul 6, 2004·American Journal of Ophthalmology·Noemi LoisFrederick W Fitzke
Sep 14, 2020·Cell Biochemistry and Biophysics·Justyna FursoTadeusz J Sarna
Nov 27, 2019·Biochimica Et Biophysica Acta. Molecular and Cell Biology of Lipids·Made Airanthi K Widjaja-Adhi, Marcin Golczak
Mar 13, 2021·Ophthalmic Genetics·Haya H Al-AniAndrea L Vincent
Apr 6, 2021·Ophthalmology Retina·Abrar K AlsalamahSawsan R Nowilaty
May 21, 2021·Clinical & Experimental Ophthalmology·Rachael C Heath Jeffery, Fred K Chen

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