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《Journal of Applied Clinical Pediatrics》 2012-20
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Application of Gas Chromatography-Mass Spectrometry Quantitative Techniques in Diagnosis of 3-Hydroxy-3-Methylglutaric Aciduria

JIANG Min-yan,LIU Li,LI Xiu-zhen,CHENG Jing,CAI Yan-na,PENG Min-zhi(Department of Endocrinology and Metabolisms,Guangzhou Women and Children Medical Center,Guangzhou Medical College,Guangzhou 510623,Guangdong Province,China)  
Objective To establish the urine 3-hydroxy-3-methylglutaric acid determination by gas chromatography-mass spectrometry(GC-MS) using urease pretreatment for the diagnosis of 3-hydroxy-3-methylglutaric aciduria(3-HMG). Methods The urinary 3-hydroxy-3-methylglutaric acid was pretreated with urease,added the internal standard(heptadecanoic acid),removed protein,dried by nitrogen,derivatized with BSTFA/TMCS(N,O-bistrimethylsilyltrifluoroacetamide/Trimethylchorosilane) and was converted into a trimethylsilyl derivative.The derivatization was measured and analyzed by GC-MS using total ion currency.The monitored ions at m/z 175 in 3-hydroxy-3-methylglutaric acid di-TMS and at m/z 363 in tri-TMS were used for quantification.The monitored ions at m/z 327 in internal standard were also used for quantification.The precision,accuracy and recovery were analyzed.Urine samples of 10 patients with metabolic acidosis,hypoglycemia without ketosis and highly suspected 3-HMG were determined. Results The limit of 3-hydroxy-3-methylglutaric acid was 6.17-1 234.56 μmol·L-1.The equation was Y=0.284 3X-0.003.The correlation coefficient was 0.998 6.The intra-assay coefficient of variation(CV) were 2.17%,7.79%,5.78%.Inter-assay CV were 4.19%,4.73%,9.24%.The recovery were 88.25%-108.47%.One patient with 3-HMG was diagnosed,and the level of 3-hydroxy-3-methylglutaric acid was 219.86 mmol·mol-1 creatinine. Conclusions Urinary levels of 3-hydroxy-3-methylglutaric acid determination by GC-MS using urease pretreatment shows high recovery,precision and accuracy.It can be used in the diagnosis of 3-HMG.
【Fund】: 国家科技部973项目(2006BAI05A07);; 广州市科技局支撑项目(2010J-E231-1)
【CateGory Index】: R725.8
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1 SU Yongheng1),ZHANG Rongjie1),YE Baoxian2)1)Laboratory of Physics and Chemistry,Henan Center for Disease Control and Prevention,Zhengzhou 450016 2)Department of Chemistry,Zhengzhou University,Zhengzhou 450001;Determination of carbendazim in vegetables and fruits by solid-phase extraction and UPLC-MS/MS[J];Journal of Zhengzhou University(Medical Sciences);2010-03
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