Marfan syndrome is the most common connective tissue disorder, with an incidence of 1 in 10,000. Marfan syndrome is autosomal dominantly inherited and associated with FBN-1 gene mutations. The alteration in the microfibril protein structure results in ocular, skeletal, and cardiovascular abnormalities, such as mitral valve prolapse and aortic root dilatation. Presented is an image of a 35 years old man with Marfan syndrome and a positive thumb sign, also known as a Steinberg sign. It means that the distal phalanx of the adducted thumb extends beyond the ulnar border of the palm.
2500 x 1568 pixels
Print Size @ 300 dpi
8 x 5 inches / 21 x 13 cm
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