A patient has been diagnosed with Marfan syndrome. Which of the following is the most common cause of death in these patients?
Correct Answer: Aoic disease
Description: Marfan syndrome Autosomal dominant Defect in an extracellular glycoprotein called fibrillin-1. Loss of fibrillin- Clinical manifestations of Marfan syndrome. Loss of structural suppo in microfibril rich connective tissue and excessive activation of TGF-b signaling. Skeletal abnormalities Unusually tall Exceptionally long extremities and long, Tapering fingers and toes. Cardiovascular lesions Mitral valve prolapse Aoic Dissection.
Category:
Pathology
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