Introduction: The first aim of this study is to define the severity of radiologic features according to mucopolysaccharidosis (MPS) type. The second aim is to compare spine radiographs with dual-energy X-ray absorptiometry (DXA) scores. Methodology: A total of 64 MPS children were enrolled between January 2017 and March 2021. Patients with a history of surgery, fracture or improper radiographs were excluded. Finally, 48 cases (20 MPS VI, 12 MPS IVA, 7 MPS IIIA, 4 MPS IIIB, 3 MPS II, 2 MPS I) were yielded. Among them, 38 had DXA performed in the same week with radiographs. Demographic and radiographic features and the hip acetabular index were noted. T12-L5 vertebral body heights were measured from lateral spine radiographs and divided by patient height. DXA measurements, bone mineral density and Z-scores were also recorded. Results: Spine and hip findings were most frequently seen in MPS VI and IVA. Oar-shaped ribs were more common in MPS VI, whereas anteromedial beaking of vertebra was predominantly seen in MPS IVA. Femoral head dysplasia is most common in MPS IVA, VI and I. The highest mean acetabular was observed in MPS I. The mean Z-score of L1-L4 vertebrae was low for MPS I (-3.8), IVA (-3.79) and VI (-3.73), but normal for MPS II (0.6) and IIIA (0.23). Correlation between the Z-score and vertebral index was highest in the L1 vertebral body. Conclusion: Interpreting the characteristic radiographic features of different MPS types is important. In addition to dysostosis multiplex, quantitative measurements from radiographs may be beneficial in evaluating disease progression.

简介：本研究的首要目的是根据粘多糖贮积症 (MPS) 类型确定放射学特征的严重程度。第二个目的是将脊柱 X 光片与双能 X 射线吸收测定法 (DXA) 分数进行比较。方法：2017 年 1 月至 2021 年 3 月期间，共有 64 名 MPS 儿童入组。排除有手术史、骨折或 X 光片不当的患者。最终产生48例（20例MPS VI、12例MPS IVA、7例MPS IIIA、4例MPS IIIB、3例MPS II、2例MPS I）。其中，38 人在同一周进行了 DXA 和 X 光片检查。注意到人口统计学和放射学特征以及髋臼指数。T12-L5椎体高度是根据侧向脊柱 X 光片测量的，并除以患者身高。还记录了 DXA 测量值、骨矿物质密度和 Z 分数。结果：脊柱和髋关节的表现在 MPS VI 和 IVA 中最常见。桨形肋骨在 MPS VI 中更常见，而椎骨前内侧喙状结构主要见于 MPS IVA。股骨头发育不良在 MPS IVA、VI 和 I 中最常见。在 MPS I 中观察到最高的平均髋臼。MPS I (-3.8)、IVA (-3.79) 和VI (-3.73)，但 MPS II (0.6) 和 IIIA (0.23) 正常。Z 分数与椎骨指数之间的相关性在 L1 椎体中最高。结论：解释不同 MPS 类型的典型射线照相特征很重要。除了多发性骨发育不全，X 光片的定量测量可能有助于评估疾病进展。