Saturday, April 11, 2009

Clinical diagnosis

Clinical diagnosis is based on the Gent criteria, which
require the presence of major diagnostic criteria in two systems,
with involvement of a third system. Major criteria include any
combination of four of the skeletal features, ectopia lentis,
dilatation of the ascending aorta involving at least the sinus of
Valsalva, lumbospinal dural ectasia detected by MRI scan, and a
first degree relative with confirmed Marfan syndrome. Minor
features indicating involvement of other symptoms include
striae, recurrent or incisional herniae, and spontaneous
pneumothorax.

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