Large Print Reviews

Bietti's Crystalline Dystrophy Resource Guide
By the National Eye Institute

Home | What's New | Reviews | Articles | Travel | Links | Search
Large Print Bookstore | Low Vision Product Store



Eye Diseases and Disorders Series - LPR Staff Project - December 27, 2004

(This article is for information purposes only. Always consult your doctor for medical advice.)

Click here to jump to a list of Bietti's Crystalline Dystrophy related links.

The following is an abridgement of an article that was provided courtesy of the National Eye Institute. A complete, printed copy of this article, as well as a other free publications offered by the NEI, can be ordered via their NEI Publications Catalog.



Bietti's Crystalline Dystrophy Resource Guide
By the National Eye Institute, National Institutes of Health

The information provided in this Resource Guide was developed by the National Eye Institute (NEI) to help patients and their families in searching for general information about Bietti's crystalline dystrophy. An eye care professional who has examined the patient's eyes and is familiar with his or her medical history is the best person to answer specific questions.

Other names

Bietti's crystalline corneoretinal dystrophy

What is Bietti's Crystalline Dystrophy?

Bietti's crystalline dystrophy (BCD) is an inherited eye disease named for Dr. G. B. Bietti, an Italian ophthalmologist, who described three patients with similar symptoms in 1937. The symptoms of BCD include: crystals in the cornea (the clear covering of the eye); yellow, shiny deposits on the retina; and progressive atrophy of the retina, choriocapillaries and choroid (the back layers of the eye). This tends to lead to progressive night blindness and visual field constriction. BCD is a rare disease and appears to be more common in people with Asian ancestry.

People with BCD have crystals in some of their white blood cells (lymphocytes) that can be seen by using an electron microscope. Researchers have been unable to determine exactly what substance makes up these crystalline deposits. Their presence does not appear to harm the patient in any other way except to affect vision.

What causes Bietti's Crystalline Dystrophy?

From family studies, we know that BCD is inherited primarily in an autosomal recessive fashion. This means that an affected person receives one nonworking gene from each of his or her parents. A person who inherits a nonworking gene from only one parent will be a carrier, but will not develop the disease. A person with BCD syndrome will pass on one gene to each of his or her children. However, unless the person has children with another carrier of BCD genes, the individual's children are not at risk for developing the disease. More about autosomal recessive inheritance.

In September 2000, NEI researchers reported that the BCD gene had been localized to chromosome #4. In this region of chromosome #4 there are hundreds of genes. Researchers are now looking for which of the genes in this region of chromosome #4 causes BCD. Finding the gene may shed light on the composition of the crystals found in the corneas of patients with BCD and on what causes the condition. More about finding disease genes.

Can Bietti's Crystalline Dystrophy Be Treated?

At this time, there is no treatment for BCD. Scientists hope that findings from gene research will be helpful in finding treatments for patients with BCD.

Resource List

The following organizations may be able to provide additional information on Bietti's crystalline dystrophy:
Related Articles & Reviews:
Back to top


About LPR | Privacy Policy | Site Map

Questions or Comments? Send an email to:
info@largeprintreviews.com

Copyright Large Print Reviews 2004 - All Rights Reserved