Zhang Huili, Zhu Yuling, Sun Yiming, Liang Yingyin, Li Yaqin, Zhang Yu, Deng Langhui, Wen Xingxuan, Zhang Cheng
Department of Neurology, First Affiliated Hospital, Sun Yat-sen University, No. 58 Zhongshan 2nd Road, Guangzhou 510080, China.
Department of Anatomy and Neurobiology, Zhongshan School of Medicine, Sun Yat-sen University, No. 58 Zhongshan 2nd Road, Guangzhou 510080, China.
Dis Markers. 2015;2015:141856. doi: 10.1155/2015/141856. Epub 2015 Mar 17.
To improve assessment of dystrophinopathy, the aim of this study was to identify whether serum creatinine (Crn) level reflects disease severity.
Biochemical, Vignos score, and genetic data were collected on 212 boys with dystrophinopathy.
Serum Crn level had a strong inverse correlation with Vignos score by simple correlation (r = -0.793) and partial correlation analysis after adjustment for age, height, and weight (r = -0.791; both P < 0.01). Serum Crn level was significantly higher in patients with in-frame than out-of-frame mutations (Z = -4.716, P < 0.01) and in Becker muscular dystrophy (BMD) patients than Duchenne muscular dystrophy (DMD) patients at ages 4, 5, 7, and 9 yr (all P < 0.0125). After adjusting for age, height, and weight, BMD patients still had a significantly higher serum Crn level than DMD patients (β = 7.140, t = 6.277, P < 0.01).
Serum Crn level reflected disease severity and may serve as a supplemental index to distinguish DMD from BMD in clinical practice.
为了改进对肌营养不良症的评估，本研究的目的是确定血清肌酐（Crn）水平是否反映疾病严重程度。
收集了212例患有肌营养不良症男孩的生化指标、Vignos评分和基因数据。
通过简单相关性分析（r = -0.793）以及在调整年龄、身高和体重后的偏相关性分析（r = -0.791；均P < 0.01），血清Crn水平与Vignos评分呈强负相关。在4岁、5岁、7岁和9岁时，框内突变患者的血清Crn水平显著高于框外突变患者（Z = -4.716，P < 0.01），贝克型肌营养不良（BMD）患者的血清Crn水平高于杜氏肌营养不良（DMD）患者（均P < 0.0125）。在调整年龄、身高和体重后，BMD患者的血清Crn水平仍显著高于DMD患者（β = 7.140，t = 6.277，P < 0.01）。
血清Crn水平反映疾病严重程度，在临床实践中可作为区分DMD和BMD的补充指标。
