脂质沉积性肌病骨骼肌磁共振31磷波谱的临床价值研究

目的探讨脂质沉积性肌病(LSM)患者骨骼肌磁共振31磷波谱(31P-MRS)改变特征,以及在LSM辅助诊断和疗效评价方面的临床价值。方法对12例LSM患者在治疗前后和11例对照者分别进行31P-MRS扫描,获取波谱图像,计算谱线中无机磷酸盐(Pi)、磷酸肌酸(PCr)及三磷酸腺苷(ATP)的峰下面积,记录Pi/ATP、PCr/ATP和Pi/PCr的比值,计算Pi、PCr、细胞内pH(pHint)、二磷酸腺苷(ADP)和磷酸化潜能(PP)的值,并比较LSM患者治疗前和对照组、LSM患者治疗前后上述31P-MRS指标的差异。结果 LSM患者治疗前的PCr、PCr/ATP和PP较对照组明显降低(P<0.05),Pi/PCr和ADP较对照组明显升高(P<0.05),Pi、Pi/ATP和pHint与对照组比较无明显差异(P>0.05);LSM患者治疗后的PCr、PCr/ATP和PP较治疗前明显升高(P<0.05),ADP较治疗前明显降低(P<0.05),Pi、Pi/ATP、Pi/PCr和pHint与治疗前比较无明显差异(P>0.05)。结论31P-MRS可无创性检测LSM患者肌肉组织的能量代谢变化,有利于LSM的辅助诊断,并可运用于LSM患者的疗效评价。

The clinical value of 31p magnetic resonance spectroscopy in patients with lipid storage myopathy

Objective To investigate the 31P magnetic resonance spectroscopy(31P-MRS)manifestations of skeletal muscle in patients with lipid storage myopathy(LSM)and evaluate its clinical value in auxiliary diagnosis and therapeutic effect.Methods 31P-MRS scanning was performed in 12 patients prior to and after treatment and 11 collators.The areas under resonance of inorganic phosphate(Pi),phosphocreatine(PCr)and adenosine triphosphate(ATP)were calculated from the 31P-MRS images,then the ratios of Pi/ATP,PCr/ATP and Pi/PCr and the values of Pi,PCr,intracellular pH(pHint),adenosine diphosphate(ADP)and phosphorylation potential(PP)were calculated at last.The above-mentioned variables were compared among LSM patients prior to treatment and the control group,and variables were also compared in LSM patients prior to and after treatment.Results Compared with the control group,LSM patients prior to treatment had significantly lower PCr,PCr/ATP and PP(P<0.05),and significantly higher Pi/PCr and ADP(P<0.05),but there were no significant differences in Pi,Pi/ATP and pHint between the control group and LSM patients prior to treatment(P>0.05).Compared with LSM patients prior to treatment,LSM patients after treatment had significantly higher PCr,PCr/ATP and PP(P<0.05),and significantly lower ADP(P<0.05),but there were no significant differences in Pi,Pi/ATP,Pi/PCr and pHint between LSM patients prior to and after treatment(P>0.05).Conclusion 31P-MRS can monitor the variations of muscle energetic metabolism in LSM patients noninvasively,which is useful for the auxiliary diagnosis and can be applied to assess the treatment response.