<unk>  <unk>  is a genetic disorder of the eyes that causes loss of vision. Symptoms include trouble seeing at night and decreasing peripheral vision (side and upper or lower visual field). As peripheral vision worsens, people may experience "tunnel vision". Complete blindness is uncommon. Onset of symptoms is generally gradual and often begins in childhood.<unk>  is generally inherited from one or both parents or rarely it can be newly evolved from a miscoding during DNA division. It is caused by genetic miscoding of proteins in one of more than 300 genes involved. The underlying mechanism involves the progressive loss of rod photoreceptor cells that line the retina of the eyeball. The rod cells secrete a neuroprotective substance (Rod-derived cone viability factor, RdCVF) that protects the cone cells from apoptosis (cell death). However, when the rod cells die, this substance is no longer provided. This is generally followed by the loss of cone photoreceptor cells. Diagnosis is by eye examination of the retina finding dark pigment deposits caused by the rupture of the underlying retinal pigmented epithelial cells, given that these cells contain a pigment known as melanin. Other supportive testing may include the electroretinogram (ERG), visual field testing (VFT), ocular coherence tomography (OCT) and DNA testing to determine the gene responsible for a person's particular type of<unk>  (now called Inherited Retinal Dystrophy (IRD). There is currently no cure for<unk> . Efforts to manage the problem may include the use of low vision aids, portable lighting, or orientation and mobility training. Vitamin A palmitate supplements may be useful to slow worsening. A visual prosthesis may be an option in certain people with severe disease. Currently there is only one FDA-approved gene therapy that is commercially available to<unk>  patients with Leber Congenital Amaurosis Type2. It replaces the miscoded<unk> E65 protein that is produced within the Retinal Pigmented Epithelium <unk> E). It has been found to effectively work in about 50% of the patients who receive the therapy. The earlier the child receives the<unk> E65 therapy the better the chances for a positive outcome. There are many other Therapies being researched at this time and hopefully will be approved in the next few years. It is estimated to affect 1 in 4,000 people.
Retinitis pigmentosa
Far-sightedness
Photic retinopathy
Chloroquine retinopathy
Corneal dystrophy
Sickle cell retinopathy
Ocular melanosis
Diabetic retinopathy