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Loeys-Dietz Syndrome More Common Than Thought

by Medindia Content Team on Aug 24 2006 8:44 PM

Loeys-Dietz syndrome, which is a genetic disorder of the connective tissue is more common than what was previously thought, according to researchers.

"It's very important to recognize this condition and distinguish it from other connective tissue disorders," said Harry Dietz, director of Johns Hopkins University School of Medicine's Center for Marfan Syndrome Research. He added that this process could save many people from an `avoidable death."

Loeys-Dietz syndrome, results in weakened aorta as well as distorted blood vessels. If the blood vessels rupture, then it could prove fatal. Patients with Loeys-Dietz syndrome usually are misdiagnosed. Sometimes this could prove fatal, the researchers said.

In the study published in New England Journal of Medicine, the researchers said surgery could usually repair the blood vessels. ``We've learned that cardiovascular surgery is very safe and therefore you should be very aggressive about going in and repairing these aneurysms before they rupture," Dietz said. ``In contrast, in people with vascular Ehlers-Danlos syndrome, you don't do surgery unless you believe death is imminent," he said.

He also said that more studies are needed to diagnose the condition and spread awareness about its prevalence.


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