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Аутосомно-домінантна неоваскулярна запальна вітреоретинопатія

ORPHA:329211· ICD-10 H35.2· Autosomal dominant neovascular inflammatory vitreoretinopathy

Визначення(English summary)

A rare, genetic, vitreoretinal degeneration characterized by a slowly progressive vitreoretinopathy with onset during the second or third decade of life. The disease initially presents as autoimmune uveitis with reduction in the b-wave on electroretinography, and progresses with development of photoreceptor degeneration, vitreous hemorrhage, cystoid macular edema, retinal neovascularization, intraocular fibrosis, secondary glaucoma, and retinal detachment leading to phthisis and complete blindness.

Поширеність
<1 / 1 000 000
Успадкування
Autosomal dominant
Вік початку
All ages