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Gardner's Syndrome

By:
Ronen Arai

Question :

What is Gardner's syndrome? I know it causes polyps in the colon and stomach, and it is hereditary.

Debbe

Answer :

Gardner's syndrome is an inherited syndrome in which thousands of polyps develop in the colon, as well as the stomach and upper intestine (duodenum). The disorder is also associated with bony tumors in the jaw and skull, and with various soft-tissue tumors (fibromas, lipomas). The polyps associated with this syndrome usually appear around age 15 and eventually lead to cancer.

This syndrome is inherited in a dominant manner, which means only one copy of the abnormal gene needs to be passed from parent to child for the disease to appear. Thus, an affected patient has a 50 percent chance of passing the disease on to his or her children.

Since the inevitable outcome of this disease is colon cancer (typically about 10-15 years after the onset of the polyps), patients with documented Gardner's should have their colon and rectum removed as a preventive measure. Although there is no recommended nonsurgical therapy for Gardner's, studies have shown that the colon polyps regress to a significant degree with use of sulindac (Clinoril), a nonsteroidal anti-inflammatory drug. Since other polyps may be present (in the stomach and duodenum), regular endoscopic examination of these areas is also prudent.

Another important issue is family screening. All blood relatives of a person diagnosed with Gardner's syndrome should be screened with colonoscopy. There are also genetic tests to screen younger patients who may have not developed the polyps yet.

 

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