Basic Defects in the Marfan Syndrome

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Abstract
The Marfan syndrome was the "founding member" of the heritable disorders of connective tissue.1 Although this nosologic category has expanded to subsume a rich diversity of diseases involving fibrous connective tissue and ground substance, the Marfan syndrome has continued to attract a disproportionate share of interest for several reasons: its relatively high prevalence; its protean manifestations, bringing patients within the purview of diverse medical specialists; its frequently catastrophic involvement of the aorta; its high association with premature mortality; its probable affliction of a venerated American president; and the occurrence of many of its somatic manifestations in other conditions and indeed . . .