粘多糖病系遗传代谢紊乱疾病,临床上少见。其特点为全身各组织细胞内及尿中有过多的粘多糖酸,具有丑陋面容,呈侏儒状,骨胳异常,运动、智力障碍及角膜混浊。按遗传方式,酶的缺陷,排出粘多糖种类和X线照片的不同分六型。其中Ⅳ型发病与近亲婚配有关,生后正常,12～18月会走时常发现异常,表现特征性骨胳改变,智力正 Mucopolysaccharidosis genetic metabolic disorders, clinically rare. It is characterized by an excess of mucopolysaccharide within the cells of the body and in the urine, with ugly face appearance, pygmy, skeletal abnormalities, sports, mental retardation and corneal opacity. By genetic means, enzyme defects, the type of mucopolysaccharide excretion and X-ray photos of different sub-six. Of which type IV incidence and related to the marriage of the relatives, normal after birth, 12 to 18 months will go often found abnormalities, the performance of characteristic skeletal changes, intelligence is