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Review
. 1993 Mar;103(3):968-70.
doi: 10.1378/chest.103.3.968.

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

H Masuda  1 , Y Morishita, A Taira, M Kuriyama
Affiliations
Review

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

H Masuda et al. 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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