New Research for Familial Exudative Vitreoretinopathy

Familial exudative vitreoretinopathy (FEVR) is an inherited retinal vascular disease, which is potentially active lifelong, and can lead to blindness by leakage into and under the retina, creating an exudative retinal detachment.   (See Sightlines, Dec. 2005 & 2010)  People born with FEVR lack blood vessels in the peripheral retina.  Children known to be at risk of having FEVR are often treated with laser that destroys the areas of the retina without blood vessels.  The disease progresses into other areas with blood vessel loss or capillary drop out.  The blood vessels are damaged and begin to leak. 
The blood vessels are tubes of endothelial cells that are held together by adhesive proteins—the best known of which are Claudins and VE Cadherins.  We have recently shown that by adding the protein Norrin to retinal endothelial cell cultures we can increase the amount of Claudin 5 and VE Cadherin.  This should reverse the leakage of blood vessels and eliminate the need for destructive laser treatments.  This is the first suggestion that drug therapy might be possible for FEVR, a goal we have been seeking for many years.  Much needs to be done to demonstrate that this can become a mainstay of FEVR therapy, but this seems like a good beginning step.

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