| Marfan syndrome is an inherited disorder that | | | | weakens is that an aneurysm may form. Marfan |
| affects connective tissue the fibers that provide | | | | syndrome can be mild to harsh, and the |
| the framework and support for your body. | | | | symptoms can vary. People with Marfan |
| Connective tissue is not a single entity, but a | | | | syndrome are frequently very tall, thin and loose |
| catch-all term for everything in your body that | | | | jointed. Most people with Marfan syndrome have |
| keeps you from falling apart. The familiar tendons | | | | heart and blood vessel problems, such as a |
| and ligaments keep bones and muscles together, | | | | weakness in the aorta or heart valves that leak. |
| but other connective tissue is more obscure, like | | | | They may also have problems with their bones, |
| the elastic fibers in the aorta that keep it soft and | | | | eyes, skin, nervous system and lungs. Different |
| rubbery. Marfan syndrome is caused by mutations | | | | people may need different types of treatment. |
| in the FBN1 gene on chromosome 15. People have | | | | Some people may not require treatment, but |
| a pair of FBN1 genes. Because it is dominant, | | | | others may need drugs that lower heart rate, |
| people who have inherited one affected FBN1 | | | | blood pressure or both. |
| gene from either parent will have Marfan's. | | | | If dural ectasia (swelling of the covering of the |
| Marfan syndrome affects all of these structures. | | | | spinal cord) develops, medication may help |
| It only happens to about 1 in every 5,000 people. | | | | minimize any associated pain. Anticoagulant |
| People with Marfan syndrome have loose tendons | | | | medications such as warfarin are needed after |
| and ligaments, less elasticity in the aorta, and | | | | artificial heart-valve placement. Genetic counseling |
| longer arms and legs. In the Marfan syndrome, | | | | and specialized clinics are available at many |
| the walls of the major arteries are weakened. | | | | academic medical centers for affected persons |
| The aorta, the main artery that leaves the heart, | | | | and family members. Annual evaluations are |
| often is affected. When this occurs, it gets bigger, | | | | important to detect any changes in the spine or |
| which can weaken the inner aortic wall. Under | | | | sternum. This is particularly important in times of |
| strenuous exertion, the aorta wall can tear. Blood | | | | rapid growth, such as adolescence. A serious |
| can leak through these tears into the aortic wall, | | | | deformity can not only be disfiguring but can also |
| separating its layers a process called aortic | | | | prevent the heart and lungs from functioning |
| dissection. | | | | properly. |
| Another problem that may occur if the aortic wall | | | | |