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KMID : 0378019770200110055
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New Medical Journal
1977 Volume.20 No. 11 p.55 ~ p.59
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Marfan Syndrome with Mitral Stenosis
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尹堅一/Yoon, Kyun Ill
全利理/李圭玉/朴利甲/Jhun, Lily/Lee, Kyu Ok/Park, Li Gap
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Abstract
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The Marfan syndrome is a heritable, generalized disorder of connective tissue, clinically manifested by abnormalities of the eye (especially ectopia lentis), of the skeletal system (especially excessive length of the long bones), and of the cardiovascular system (especially diffuse and/or dissecting aneurysm of the ascending aorta). This report describes a 27 year old Korean man who had Marfan syndrome associated with pure mitral stenosis, severe aortic regurgitation, aneurysm of the ascending and the abdominal aorta. Family pedigree of this case demonstrated heritable nature and it´s transmission as a abdominal trait. He showed typical arachnodactyly with abnormal skeletal ratio and dolichocephaly, high. arched palate, also. There was no optical involvement. The mitral stenosis was diagnosed successfully with cardiac catheterization. The aortic regurgitation and aneurysm of the ascending aorta and the abdominal aorta were discovered by cineangiography.
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KEYWORD
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