Marfan syndrome (or Marfan's syndrome) is a genetic disorder of the connective tissue. People with Marfan's are typically tall, with long limbs and long thin fingers. The most serious complication is defects of the heart valves and aorta. It may also affect the lungs, eyes, the dural sac surrounding the spinal cord, skeleton and the hard palate.
Treatment of Marfan's syndrome
There is no cure for Marfan syndrome, but life expectancy has increased significantly over the last few decades, and clinical trials are underway for a promising new treatment. The syndrome is treated by addressing each issue as it arises, and, in particular, considering preventive medication, even for young children, to slow progression of aortic dilation.
Research in laboratory mice has suggested that the angiotensin II receptor antagonist losartan, which appears to block TGF-beta activity, can slow or halt the formation of aortic aneurysms in Marfan syndrome. A large clinical trial sponsored by the National Institutes of Health comparing the effects of losartan and atenolol on the aortas of Marfan patients was scheduled to begin in early 2007, coordinated by Johns Hopkins.
Examine the eyes and mouth of your child or young teenager. Many individuals diagnosed with Marfan syndrome have crowded teeth and a "high palate" in the mouth. Usually these children are also near-sighted (myopia) and sometimes suffer from a dislocated lens in the eye, which makes the eye look upward.
The Marfan syndrome is inherited and affects many parts of the body. The disorder affects males and females from all racial and ethnic groups. Marfan syndrome is associated with incomplete penetrance; therefore not all persons carrying the mutation develop the disease. It is one of the most common of the more than 100 inherited disorders of connective tissue (material that holds tissues of the body together).
The above article provides detailed description about Marfan's Syndrome. It is important to know about this disease and its symptoms.
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