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Wednesday, May 16, 2018

STARGARDT'S MACULAR DYSTROPHY

Stargardt disease is an inherited disorder of the retina. For most people, vision loss progresses slowly over time to 20/200 or worse. (Normal vision is 20/20).
People with the disease might notice gray, black, or hazy spots in the center of their vision, or that it takes longer than usual for their eyes to adjust when moving from light to dark environments. Their eyes may be more sensitive to bright light. Some people also develop color blindness later in the disease.
Autosomal recessive mutations account for about 95 percent of Stargardt disease. 
Currently, there is no treatment for Stargardt disease. Some doctors encourage people with Stargardt disease to wear dark glasses and hats when out in bright light to reduce the buildup of lipofuscin. Cigarette smoking and second hand smoke should be avoided. Supplements containing more than the recommended daily allowance of vitamin A should be avoided. There is no need to worry about getting too much vitamin A through food.
Stem cell-based therapies are showing promise for Stargardt disease in clinical trials. A U.S. company called Advanced Cell Technology (ACT) is conducting a trial of retinal pigment epithelium (RPE) cells for AMD and Stargardt disease. 

Below are photos of the macula of a 60 year old female with Stargardt's Disease.  Fortunately, her visual acuity has stabilized at 20/40.  Autofluorescence images are included.  



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