Cardiac structural involvement in mucopolysaccharidoses
Cardiac structural involvement in mucopolysaccharidoses
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DOI：
10.1159/000064674
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发表时间：
2002-01-01
期刊：
影响因子：
1.9
通讯作者：
Segni, G
中科院分区：
文献类型：
--
作者：
Rigante, D;Segni, G
粘多糖症（MPS）是由于糖胺聚糖降解受损而引起的溶酶体储存障碍。大多数MPS患者存在心脏受累，但其临床影响尚未确定。对39例4-22岁MPS患者的心血管异常进行了评价。检测到瓣膜病变和不同形式的心脏受累。最常见的病变是二尖瓣增厚伴返流或狭窄，与MPS类型无关。二尖瓣增厚23例，主动脉瓣增厚11例，充血性心力衰竭1例。MPS I型和II型的变化最严重。对每一位患有MPS的患者都应进行常规的全面心脏病学检查。版权所有（C）2002 S. Karger AG，巴塞尔。
Mucopolysaccharidoses (MPS) are lysosomal storage disorders due to impaired glycosaminoglycan degradation. Cardiac involvement is present in most patients with MPS although its clinical impact is still undetermined. Cardiovascular abnormalities were evaluated in 39 patients with MPS aged 4-22 years. Valvular lesions and different forms of cardiac involvement were detected. The most common lesion was thickening of the mitral valve with regurgitation or stenosis, regardless of the MPS type. Mitral valve thickening was observed in 23 patients, aortic valve thickening in 11 patients and congestive heart failure in only 1 patient with MPS Ill. The most severe changes were registered for MPS types I and II. Complete cardiological investigation should be routinely warranted in every patient inflicted with MPS. Copyright (C) 2002 S. Karger AG, Basel.