粘多糖贮积症的X线诊断及其临床表现  

X-ray diagnosis and clinical manifestations of mucopolysaccharidosis

作　　者：宋亚峰[1] 何荷花[1] 徐霖[1] 

机构地区：[1]湖北十堰郧阳医学院附属太和医院放射科,湖北十堰442000

出　　处：《罕少疾病杂志》2006年第5期29-31,共3页Journal of Rare and Uncommon Diseases

摘　　要：目的探讨粘多糖贮积症的X线诊断要点。方法回顾分析粘多糖贮积症的影像特点及临床表现。结果异常的粘多糖可沉积于体内不同的组织和器官,形成不同类型的粘多糖贮积症,各型的影像和临床表现均有一定特征。粘多糖在骨组织沉积可致成骨发育障碍和变形,在关节沉积可引起关节硬化;在动脉壁沉积可形成假粥样硬化斑;在脑组织沉积致使智力低下。结论粘多糖贮积症的影像表现有一定的特异性,但进一步准确分型应对尿中粘多糖类型及组织细胞培养作酶学分析或进行基因密码检测。Objective To probe criteria for diagnosing mucopolysaccharidosis using X-ray. Methods Imaging characteristics of mucopolysaccharidosis on X-ray films were reviewed and their clinical manifestations were discussed. Results Abnormal mucopolysaccharides can deposit in a wide variety of body organs causing mucopolysaccharidosis in different types, which present unique X-ray features and clinical signs. Deposition of mucopolysaccharides in bone tissue causes disorder in bone development and deformation of bone; in joint causes hardened joint; in artery wall causes pseudo-plaque of atherosclerosis; and in brain causes poor intelligence. Conclusion Mucopolysaccharidosis has characteristic findings in X-ray films. However, further classification of it depends on identifying sorts of mucopolysaccharides by urine test, cell culture, enzyme analysis and gene code testing.

关 键 词：粘多糖贮积症 X线诊断 

分 类 号：R363[医药卫生—病理学]