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Article
October 1980

Dominantly Inherited Macular Dystrophy With Flecks (Stargardt)

Author Affiliations

From the Children's Mercy Hospital Departments of Ophthalmology (Dr Cibis) and Genetics (Ms Morey and Dr Harris), University of Missouri School of Medicine, Kansas City.

Arch Ophthalmol. 1980;98(10):1785-1789. doi:10.1001/archopht.1980.01020040637010
Abstract

• A family had dominantly inherited macular dystrophy with flecks and without evidence of major cone dysfunction. This family shows that the clinical picture of Stargardt's disease can result from at least two different genes: one with dominant and another with recessive heredity. Some patients had fundus flavimaculatus flecks of Stargardt's disease; others did not. In our opinion progressive atrophic macular (foveal) dystrophy as Stargardt's disease without flecks is not a separate clinical entity but part of the same genetic defect. Furthermore, a dominant hereditary pattern in patients without flecks or major cone dysfunction is insufficient evidence for classification of such patients as having cone dystrophy.

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