Renovascular hypertension in Marfan syndrome |
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Authors: | Michelle A Baum H William Harris Patricia E Burrows Deborah E Schofield Michael J G Somers |
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Institution: | (1) Division of Nephrology, Children’s Hospital, Harvard Medical School, Boston, Massachusetts, USA, US;(2) Department of Radiology, Children’s Hospital, Harvard Medical School, Boston, Massachusetts, USA, US;(3) Department of Pathology, Children’s Hospital, Harvard Medical School, Boston, Massachusetts, USA, US |
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Abstract: | A defect in fibrillin integrity predisposes patients with Marfan syndrome to vascular wall abnormalities, most notably aortic
rupture and dissection. Renal vascular anomalies have not been described previously in children with Marfan syndrome. In this
report, we detail data from a hypertensive 14-year-old girl with clinical stigmata of Marfan syndrome and a diagnostic evaluation
significant for characteristic aortic root dilatation and aneurysm, as well as a disparity in renal size and function exacerbated
by captopril administration. Renal arteriography confirmed a left main renal artery stenosis that was not amenable to balloon
angioplasty. Surgical resection resulted in significant improvement in hypertension. Pathological examination of the resected
renal artery segment revealed intimal proliferation, fragmentation of the elastic media, and inner medial dissection. This
patient demonstrates that, in addition to the aorta, renal arteries can be affected with the characteristic vascular wall
pathology of Marfan syndrome, resulting in systemic hypertension. These data suggest that children with Marfan syndrome and
hypertension need to be evaluated carefully for the presence of renal anomalies.
Received October 15, 1996; received in revised form and accepted January 10, 1997 |
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Keywords: | : Marfan syndrome Hypertension Renal artery stenosis Fibrillin Arteriography Angioplasty |
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