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Aortic stenosis associated with Scheie's syndrome. Report of successful valve replacement.

Author(s): Deborah Taira, Y Morishita, M Kuriyama, H Masuda

Publication date: 1993-02-28

Journal: Chest

Keywords: Aortic Valve, Aortic Valve Stenosis, diagnosis, genetics, surgery, Heart Valve Prosthesis, Humans, Male, Middle Aged, Mucopolysaccharidosis I

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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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PubMed ID:: 8449111

Keywords: Aortic Valve,Aortic Valve Stenosis,diagnosis,genetics,surgery,Heart Valve Prosthesis,Humans,Male,Middle Aged,Mucopolysaccharidosis I

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Keywords: Aortic Valve, Aortic Valve Stenosis, diagnosis, genetics, surgery, Heart Valve Prosthesis, Humans, Male, Middle Aged, Mucopolysaccharidosis I

Aortic stenosis associated with Scheie's syndrome. Report of successful valve replacement.

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