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目的分析粘多糖病Ⅰ型的X线、CT表现,提高对本病的认识。方法回顾分析粘多糖病Ⅰ型的X线表现特点和CT检查及图像后处理表现。结果粘多糖病Ⅰ型具有较典型的骨骼X线改变,(1)头大,蝶鞍呈乙型;(2)掌指骨粗短;(3)上、下肢骨中央膨胀两端变细;(4)脊椎后凸成角,后凸椎体前下缘成鸟嘴状突出,上下椎体呈卵圆形;(5)肋骨增宽呈船桨;(6)髋臼变浅,髋臼角增大;(7)股骨颈增长,髋内翻或外翻。结论粘多糖病Ⅰ型的影像表现有一定的特异性,X线照片、CT检查及图像后处理技术有利于提高粘多糖病Ⅰ型的诊断水平。
Objective To analyze the X-ray and CT findings of mucopolysaccharidosis type Ⅰ and to improve the understanding of the disease. Methods The X-ray features, CT examination and image post-processing of mucopolysaccharidosis type Ⅰ were retrospectively analyzed. Results The mucopolysaccharidosis type Ⅰ had more typical skeletal X-ray changes, (1) the head was large and the sella was B type; (2) the metacarpal phalanges were stubby; (3) 4) the kyphosis angle, kyphotic anterior lower edge of the convex edge of the bill of mouth, the upper and lower vertebral body was oval; (5) ribs widened was oars; (6) acetabular shallow, acetabular angle Increase; (7) femoral neck growth, hip varus or eversion. Conclusions Mucopolysaccharidosis type Ⅰ has certain specificity. X - ray, CT and image postprocessing techniques are helpful to improve the diagnosis of type Ⅰ mucopolysaccharidosis.