$Unique_ID{BRK00458}
$Pretitle{Ill Defined Symptoms and Rare Diseases}
$Title{What is Stargart's Disease?}
$Subject{eyes blindness Stargart's vision}
$Volume{N-21}
$Log{}

Copyright (c) 1991   Tribune Media Services, Inc.


What is Stargart's Disease?


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QUESTION:  My 15 year old son was just diagnosed as having Stargart's Disease.
He is legally blind in his left eye and the retina specialist is watching his
right eye.  There is some scar tissue here.  Can you tell me about this
disease?

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ANSWER:  The disease is also know as Juvenile Macular Degeneration and was
first described by Dr. F. Stargardt in a German ophthalmological journal in
1909.  It comes on between 6 and 20 years of age in children who have been
normal until then, and is marked by a gradual decrease of vision in both eyes.
At first there are no signs on examination of the retina, but then changes in
the pigmented epithelium at the back of the eye occur, assuming a "beaten
bronze" atrophy.  It is this defect in the pigmented epithelium that causes
the vision loss.  It is an inherited disease, normally a recessive gene (not
showing in each individual), but may be dominant in some families.  Gradually
the visual elements in the area of high vision (the macular) and the areas
that surround it (the peri-macular area) disappear, making this condition a
gradually progressive one.  At present there are no effective therapies
available, but you seem to have the treatment in appropriate hands, and should
new developments occur, a Retinal Specialist would be the first to be aware of
new treatments.  Current research in the transplantation of healthy retinal
cells into eyes suffering from inherited retinal degeneration has been
successful in rats, a break through, but still a long way from use in humans.
The work is being carried on by Drs.  James E. Turner and Linxi Li at the
Bowman Gray School of Medicine, Winston Salem, N.C., and provides the first
ray of hope for suffers of inherited retinal degenerative disease.
ÿ
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The material contained here is "FOR INFORMATION ONLY" and should not replace 
the counsel and advice of your personal physician.  Promptly consulting your 
doctor is the best path to a quick and successful resolution of any medical 
problem.
