Renal aery stenosis is not associated with:March 2009
Ans. A: Marfan's syndromeThe most common cause of renal aery stenosis is an atheromatous plaque.Marfan Syndrome:Marfan syndrome (MFS) is a spectrum of disorders caused by a heritable genetic defect of connective tissue that has an autosomal dominant mode of transmission.The defect itself has been isolated to the FBN1 gene on chromosome 15, which codes for the connective tissue protein fibrillin. Abnormalities in this protein cause a myriad of distinct clinical problems, of which the musculoskeletal, cardiac, and ocular system problems predominate.The skeleton of patients with MFS typically displays multiple deformities including arachnodactyly (ie, abnormally long and thin digits), dolichostenomelia (ie, long limbs relative to trunk length), pectus deformities (ie, pectus excavatum and pectus carinatum), and thoracolumbar scoliosis.Finally, blindness may result from unrecognized and untreated glaucoma, retinal detachment, and cataracts.In the cardiovascular system, aoic dilatation, aoic regurgitation, and aneurysms are the most worrisome clinical findings. Mitral valve prolapse that requires valve replacement can occur as well.Ocular findings include myopia, cataracts, retinal detachment, and superior dislocation of the lens.Takayasu aeritis is also known as pulseless disease because of frequent occlusion of aeries arising from the aoa.It may also occlude renal aeries.Polyaeritis nodosa is a multisystem necizing vasculitis of small and medium sized muscular aeries in which involvement of the renal and visceral aeries is characteristic.There is fibrinoid necrosis with compromise of the lumen.
Marfan's syndrome
atherosclerosis
polyaeritis nodosa
takayasu aeritis