Clinical Considerations: Marfan Syndrome
- Marfan syndrome is a connective-tissue disorder caused by mutations in fibrillin genes.
- Fibrillin is critical for elastic fibers, so fibrillin mutations can weaken tissues that depend on elastic recoil and structural support.
- Long slender limbs and long fingers are typical body features described in Marfan syndrome.
- Lens dislocation occurs when the eye lens shifts out of its normal position, and it is a common Marfan syndrome problem.
- Cardiovascular problems in Marfan syndrome are important because the aorta and other connective-tissue-rich structures depend on strong elastic fibers.
- Aortic rupture can cause death because the aorta is the major artery carrying blood from the heart to the body.
- Blood-pressure lowering drugs and aortic replacement surgery are management approaches used to reduce risk in severe cardiovascular involvement.
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