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计算机断层扫描在黏多糖贮积症患者中的价值。

The value of computed tomography in patients with mucopolysaccharidosis.

作者信息

Nelson J, Grebbell F S

机构信息

Department of Medical Genetics, Royal Victoria Hospital, Belfast, Northern Ireland.

出版信息

Neuroradiology. 1987;29(6):544-9. doi: 10.1007/BF00350438.

Abstract

Cranial computed tomography (CT) was performed on 11 cases of mucopolysaccharidosis (MPS) IVA (Morquio syndrome). Our results suggest that although the patients may have normal intelligence CT changes may be seen with increasing age. In two cases white matter low density was found and in a third there was gross dilatation of the ventricles, basal cisterns and subarachnoid space. Nine other patients with various types of mucopolysaccharidosis also had cranial CT performed and in general those types associated with mental retardation showed changes although there was an interesting exception involving a case of MPS IIIA who had a normal CT scan.

摘要

对11例黏多糖贮积症IVA型(Morquio综合征)患者进行了头颅计算机断层扫描(CT)。我们的结果表明,尽管患者智力可能正常,但随着年龄增长,CT检查可能会出现改变。2例患者发现白质低密度,第3例患者脑室、脑基底池和蛛网膜下腔明显扩张。另外9例患有各种类型黏多糖贮积症的患者也进行了头颅CT检查,总体而言,那些与智力发育迟缓相关的类型显示出改变,不过有一个有趣的例外,一名MPS IIIA型患者的CT扫描结果正常。

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