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目的比较儿童戈谢病(Gaucher disease,GD)治疗前后脊柱椎体骨密度(bone mineral density,BMD)的变化。方法 30例确诊的GD患儿,其中男18例,女12例,年龄4～17岁,平均年龄11.2±4.1岁,其中5例进行脾脏切除。在规范酶替代治疗和治疗9月后在常规随访CT扫描时,同时采用定量CT(quantitative computed tomography,QCT)测定T12-L4间无明显变形及病理性骨折的椎体骨密度,取各椎体平均骨密度。采用配对t检验比较治疗前后患儿骨密度变化。结果 30例患儿治疗前椎体平均骨密度值176.2±20.0 mg/cm3,治疗9月后平均骨密度值为197.8±18.1 mg/cm3,较治疗前椎体骨密度平均升高12.3%(P<0.01);其中5例脾切除患儿治疗前后平均骨密度值分别为163.7±5.7 mg/cm3和190.3±5.2 mg/cm3,椎体骨密度较治疗前升高13.8%。结论使用QCT骨密度测量技术可以精确敏感的评估患儿骨密度情况及治疗疗效的反应,指导临床进一步治疗方案。
Objective To compare the changes of vertebral bone mineral density (BMD) before and after treatment of children with Gaucher disease (GD). Methods 30 cases of diagnosed GD children, including 18 males and 12 females, aged 4 to 17 years, mean age 11.2 ± 4.1 years, of which 5 cases of splenectomy. During the routine follow-up CT scan after the regular enzyme replacement therapy and treatment, the vertebral body BMD of T12-L4 without obvious deformation and pathological fracture was measured by quantitative computed tomography (QCT) Average bone density. Paired t test was used to compare the changes of bone mineral density in children before and after treatment. Results The average BMD of the 30 vertebral bodies before treatment was 176.2 ± 20.0 mg / cm3. After 9 months of treatment, the average BMD was 197.8 ± 18.1 mg / cm3, an average increase of 12.3% (P <0.01). The average BMD of the five cases before and after splenectomy were 163.7 ± 5.7 mg / cm3 and 190.3 ± 5.2 mg / cm3 respectively, and the vertebral bone density increased by 13.8%. Conclusions QCT bone mineral density measurement can be used to accurately and sensitively evaluate the bone mineral density in children and respond to the curative effect and guide clinical further treatment.