Review
doi: 10.1378/chest.103.3.968.
Aortic stenosis associated with Scheie's syndrome. Report of successful valve replacement
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- PMID: 8449111
- DOI: 10.1378/chest.103.3.968
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Review
Aortic stenosis associated with Scheie's syndrome. Report of successful valve replacement
Chest.
1993 Mar.
Abstract
A 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.
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