Keyfi Fatemeh, Lukacs Zoltan, Varasteh Abdolreza
Varastegan Institute for Medical Sciences, Mashhad, Iran.
Pardis Clinical and Genetic Laboratory, Mashhad, Iran.
Rep Biochem Mol Biol. 2017 Oct;6(1):40-50.
Organic acids refer to a family of compounds that are intermediates in a variety of metabolic pathways. Many organic acids are present in urine from clinically normal individuals. Elevated levels of urine organic acids cause to the organic acidurias, disorders in which some metabolic pathways in organic acid metabolism are blocked. The present work identified major and minor urinary acidic metabolites in normal subjects, and their quantitative ranges in a pediatric population of Iran.
Two hundred and fifty-one healthy subjects, including 132 males and 119 females, from 2 days to 15 years of age were enrolled. Urinary organic acids were extracted from urine with organic solvents and identified and quantified by gas chromatography-mass spectrometry.
The results provide a foundation on which to check results for patients with potentially abnormal organic acidurias. By this method 98 organic acids were identified in a pediatric population of Iran.
The present work identifies and quantifies major and minor urinary metabolites excreted by normal subjects. We also analyzed urine from 30 patients with organic acid metabolism abnormalities and compared the concentrations of specific organic acids with those from urines of normal individuals.
有机酸是指在多种代谢途径中作为中间体的一类化合物。许多有机酸存在于临床正常个体的尿液中。尿液中有机酸水平升高会导致有机酸尿症,即有机酸代谢中的某些代谢途径受阻的病症。本研究确定了正常受试者尿液中的主要和次要酸性代谢产物,以及它们在伊朗儿科人群中的定量范围。
招募了251名健康受试者,包括132名男性和119名女性,年龄在2天至15岁之间。用有机溶剂从尿液中提取尿有机酸,并用气相色谱-质谱法进行鉴定和定量。
这些结果为检查可能患有异常有机酸尿症患者的结果提供了基础。通过这种方法,在伊朗儿科人群中鉴定出98种有机酸。
本研究鉴定并定量了正常受试者排泄的主要和次要尿代谢产物。我们还分析了30例有机酸代谢异常患者的尿液,并将特定有机酸的浓度与正常个体尿液中的浓度进行了比较。