Starfardt Disease

PATHOLOGY

The Stargardt disease is the most common form of dystrophy with juvenile onset, characterized by yellow round or pisciform spots around the macula at the level of the retinal pigmented epithelium (RPE). It is associated to the accumulation of fluorescent lipofuscin, in particular A2E (bis retinoid pyridinium salt N-retilynidene N- retinylethanolamine), the major component of lipofuscin.

TREATMENT

No approved treatment currently exists for the Stargardt disease. However, clinical studies are currently in an advanced stage for this pathology for the development of drugs or gene therapies, which are already being tested on man.  The individuals with this condition should wear spectacles or sunglasses which block 100% of UV light for reducing the possibility of further damage to the eyes caused by the sun. Studies conducted on animals have evidenced that the taking of excessive quantities of vitamin A and beta carotene could promote a further accumulation of lipofuscin, as well as a toxic derivative of vitamin A called A2E; it is, therefore, advisable in general that these molecules are avoided by individuals suffering from Stargardt disease.

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