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Jpn Circ J. 1998 Sep;62(9):700-3.

Surgical treatment for Scheie's syndrome (mucopolysaccharidosis type I-S): report of two cases.

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Department of Cardiovascular Surgery, Japanese Red Cross Society, Wakayama Medical Center, Wakayama-city.


Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic lysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was carried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year-old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Scheie's syndrome associated with valvular and coronary artery disease is also reviewed.

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