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粘多糖病(Mucopolysaccharidosis简称MPS)于1952年首先由Brante提出.本病并非罕见.仅1978~1981年间国内文献报道170余例之多,均着重临床与x线表现;有关脑CT扫描改变尚未见文献报告.本文提供一例如下.男,??岁.因前囟未闭、头大来诊.一年余前其母发现患儿头大、智力低下;一年前见上腰段脊柱后突.无恶心、呕吐、头痛及头颅外伤史.体检;身高89cm,体重15kg,头围54cm.前囟未闭,扁鼻梁,牙稀小、轻度鸡胸,心尖区Ⅲ/6级收缩期杂音,肝肋下5cm,脾肋下
Mucopolysaccharidosis (MPS) was first proposed by Brante in 1952. This disease is not uncommon, with more than 170 cases reported in the domestic literature from 1978 to 1981, all of which focus on clinical and x-ray findings. Changes in brain CT scan have not been seen Reported in the literature. This article provides an example of the following. Male, aged. Because of the anterior fontanel not closed, the first large consultation .One year before her mother found that the first child with large head, mental retardation; a year ago to see the upper lumbar kyphosis No nausea, vomiting, headache and history of head trauma Physical examination Height 89cm, weight 15kg, head circumference 54cm Frontal fontanelle, flat nose bridge, sparse teeth, mild chicken chest, apex systolic murmur III / Liver ribs 5cm, spleen ribs