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| 儿童戈谢病脊柱定量cT骨密度测定随访研究 |
| Vertebral bone mineral density follow-up in children with gaucher disease with QCT |
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| DOI： |
| 中文关键词: 戈谢病 骨质稀疏 儿童 定量CT |
| 英文关键词:Gaucher disease 0sLeopenia PediaLric QuanLiLaLive CT |
| 基金项目: |
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| 中文摘要: |
| 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 densiLy，BMD)的变化。方法30例确诊的郧阅患儿，其中男18例，女12例，年龄4耀17岁，平均年龄11.2依 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(择uanLiLaLive com责uLed Lomography , 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。采用配对贼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依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豫(孕<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 |
| 英文摘要: |
| Objective To foUow-up Lhe bon^ mineral densiLy ( BMD ) of verLebrae using quanLiLaLive CT in Gaucher disease ( GD ) children wiLh enzyme rep造acemenL Lherapy. Methods 30 GD paLienLs^ male 18， remaU2 )w iLh mean age 11.2 ± 4. 1 years， ranging 4-17 years，were LreaLed w i Lh enzyme rep造acemenL Lherapy and 5 cases had sp造enecLomy. WiLh Lhe rouLine CT scan daLaseL for moniLor Lhe progression of Lhe disease， Lhe spine BMD was measured wiLh QCT aL baseline and 9 monLhs afLer LreaLmenL. Paired sLudenL L LesL was perfomied Lo compare Lhis diiTerence. Results The mean BMD of 30 GD paLienLs was 176. 2 土 20. 0 mg/cm3 aL Lhe baseline and 197. 8 土 18. 1 mg/cm3 afLer 9 monLhs LreaLmenL， which represenLed an average of 12. 3%' increase in BMD (孕 < 0. 01 ). In parLicular Lhe average BMD in Lhe children wiLh spienecLomy aL baseline was 163. 7 ±5. 7 mg/cm3 versus Lhe 190. 3 ±5. 2 mg/cm3 of BMD afLer LreaLmenL， and which was increased abouL 13. 8% .Conclusions Bone mineral densiLy measuremenL using QCT is very useful Lo moniLoring Lhe LreaLmenL effecL in children wiLh Gaucher disease. |
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