磁共振定量测定儿童及青少年戈谢病肝脾脏体积及脂质含量

目的 探讨MRI定量分析儿童及青少年戈谢病患者肝脏、脾脏体积及脂质含量的价值。方法 对经骨髓穿刺证实的42例戈谢病患者（其中12例已行脾脏切除）行腹部MR检查，序列为冠状位三维梯度回波Dixon水脂分离（3D FFE mDIXON）和T2WI-STIR序列。所有患者均正在接受酶替代治疗。评估患者肝脏、脾脏脂质含量的差异及相关性。结果 42个肝脏校正的体积值为25.15~48.99 ml/kg，平均（33.66±6.03）ml/kg，脂质含量4.63%~10.09%，平均（5.90±1.10）%；30个脾脏校正的体积值为4.48~56.04 ml/kg，平均（16.36±10.65）ml/kg，脂质含量4.78%~19.39%，平均（6.63±2.62）%。同一患者脾脏脂质含量高于肝脏（t=2.16，P=0.04）。结论 采用MRI测量肝脾脏体积及脂质含量具有一定的可行性，可用于监测儿童及青少年戈谢病患者的病情及治疗效果。

MRI in quantitative analysis of the volume and lipid content of liver and spleen in child and adolescent patients with gaucher disease

Objective To explore the value of MRI in quantitative analysis of the volume and lipid content of liver and spleen in child and adolescent patients with gaucher disease (GD). Methods Totally 42 patients with GD confirmed by bone marrow biopsy underwent abdominal MR examination. Twelve patients had undergone total splenectomy. All patients had undergone the enzyme replacement therapy. MRI was performed with coronal 3D FFE mDIXON and T2WI-STIR sequences. The volume of liver and spleen was measured on T2WI-STIR sequence images by home-made semi-automatic software. The differences and correlation in volume and lipid content of liver and spleen was analyzed. Results The mean corresponding volume index of total 42 liver was (33.66±6.03)ml/kg, range of 25.15-48.99 ml/kg, and lipid content value range of 4.63%-10.09%, the average was (5.90±1.10)%. The corresponding volume index of total 30 spleen was (16.36±10.65)ml/kg, range of 4.48-56.04 ml/kg, and lipid content range of 4.78%-19.39%, the average was (6.63±2.62)%. The lipid content of spleens was higher than that of livers (t=2.16, P=0.04). Conclusion It is feasible that MRI in quantitative analysis of the volume and lipid content of liver and spleen, and MRI can be used to monitor the progression and effect of the treatment for children and adolescent patients with GD.