| Literature DB >> 8449111 |
H Masuda1, Y Morishita, A Taira, M Kuriyama.
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.Entities:
Mesh:
Year: 1993 PMID: 8449111 DOI: 10.1378/chest.103.3.968
Source DB: PubMed Journal: Chest ISSN: 0012-3692 Impact factor: 9.410