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Babinski-Nageotte Syndrome to Burnett Syndrome -  

Bietti Disease
Lecture 40 of 77  NEXT»

(Bietti Marginal Crystalline Dystrophy)

General: Autosomal recessive; metabolic disturbance; histopathologic studies demonstrated advanced panchorioretinal atrophy with crystals and complex lipid inclusions seen in choroidal fibroblasts.
Ocular: Marginal corneal crystalline dystrophy with retinitis punctata albescens; panchorioretinal atrophy.
Clinical: Asymptomatic.

Bernauer W, Daicker B. Bietti's corneal-retinal dystrophy, a 16-year progression. Retina 1992; 12:18-20.
Kaiser-Kupfer MI, et al. Clinical biochemical and pathologic correlations in Bietti's crystalline dystrophy. Am J Ophthalmol 1994; 118:569-582.
Mauldin WM, O'Connor PS. Crystalline retinopathy (Bietti tapetoretinal degeneration without marginal corneal dystrophy). Am J Ophthalmol 1981; 92:640-646.
Welch RB. Bietti's tapetoretinal degeneration with marginal corneal dystrophy crystalline retinopathy. Trans Am Ophthalmol Soc 1977; 75:164-179.


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