Aortic Root Dilatation in Mucopolysaccharidosis I–VII
نویسندگان
چکیده
منابع مشابه
Aortic Root Dilatation in Mucopolysaccharidosis I–VII
The prevalence of aortic root dilatation (ARD) in mucopolysaccharidosis (MPS) is not well documented. We investigated aortic root measurements in 34 MPS patients at the Children's Hospital of Orange County (CHOC). The diagnosis, treatment status, age, gender, height, weight and aortic root parameters (aortic valve annulus (AVA), sinuses of Valsalva (SoV), and sinotubular junction (STJ)) were ex...
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Mucopolysaccharidosis I (MPS I), known as Hurler syndrome in the severe form, is a lysosomal storage disease due to alpha-L-iduronidase (IDUA) deficiency. It results in fragmentation of elastin fibers in the aorta and heart valves via mechanisms that are unclear, but may result from the accumulation of the glycosaminoglycans heparan and dermatan sulfate. Elastin fragmentation causes aortic dila...
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ژورنال
عنوان ژورنال: International Journal of Molecular Sciences
سال: 2016
ISSN: 1422-0067
DOI: 10.3390/ijms17122004