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Aortic stenosis associated with Scheie s syndrome Report of successful valve replacement Masuda H; Morishita Y; Taira A; Kuriyama MChest 1993[Mar]; 103 (3): 968-70A 62-year-old man who had aortic stenosis associated with Scheie's syndrome (mucopolysaccharidosis [MPS], type I-S) successfully underwent aortic valve replacement. The composition of acidic glycosaminoglycans (acid mucopolysaccharides) of the excised aortic valve analyzed by high-performance liquid chromatography (HPLC) supported the diagnosis of Scheie's syndrome. This article reviews the literature on aortic stenosis in MPS, a rare inherited metabolic disorder, and discusses biochemical features and surgical repair.|*Heart Valve Prosthesis[MESH]|Aortic Valve[MESH]|Aortic Valve Stenosis/diagnosis/genetics/*surgery[MESH]|Humans[MESH]|Male[MESH]|Middle Aged[MESH]|Mucopolysaccharidosis I/diagnosis/genetics/*surgery[MESH] |
