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目的采用磁共振质子波谱成像(1H-MRS)研究苯丙酮尿症(phenylketonuria,PKU)患儿脑白质内病灶的代谢特点,并观察不同白质病变区代谢的变化。资料与方法对8例临床确诊的PKU患儿及性别、年龄相匹配的8名健康儿童(对照组)分别行T1WI自旋回波序列(SE)、T2WI快速自旋回波序列(FSE)及液体衰减反转恢复序列(FLAIR)检查。1H-MRS检查选择侧脑室后角T2WI异常信号区及放射冠正常白质为兴趣区(ROI),测量1H-MRS谱线上氮-乙酰天门冬氨酸(NAA)、胆碱(Cho)及肌酸(Cr)等主要共振峰的面积,并计算下列化合物的比值:NAA/Cr、Cho/Cr、1000*NAA(Cho、Cr)/H2O。结果在T2WI上,所有未经治疗的PKU患儿侧脑室后角周围白质均可见非占位性、斑片状、条带状高信号。1H-MRS显示未经治疗及治疗后PKU白质异常区及放射冠诸代谢产物与正常对照组差异无统计学意义(P>0.05)。结论 MRS可作为无创性研究PKU病理生理过程的方法。
Objective To investigate the metabolic characteristics of white matter lesions in phenylketonuria (PKU) patients by 1H MRS and observe the changes of metabolism in different white matter lesions. Materials and Methods Eight healthy children with PKU and 8 healthy children (control group) with definite age were enrolled in this study. T1WI spin echo sequence (SE), T2WI fast spin echo sequence (FSE) and liquid attenuation Reverse Recovery Sequence (FLAIR) test. 1H-MRS examinations were performed to detect the T2WI abnormal signals in the posterior horn of the lateral ventricle and the normal white matter of the coronal crowns as the ROI. The levels of NAA, Cho, (Cr) and other major resonance peak area, and calculate the ratio of the following compounds: NAA / Cr, Cho / Cr, 1000 * NAA (Cho, Cr) / H2O. Results On T2WI, all untreated children with PKU showed non-site-specific, patchy, and stripe-like hyperintensities around the posterior horn of the lateral ventricle. The results of 1H-MRS showed that there was no significant difference between the untreated and untreated group (P> 0.05). Conclusion MRS can be used as a noninvasive method to study the pathophysiology of PKU.