Fatemeh Keyfi - Varastegan Institute for Medical Sciences, Mashhad, Iran - Pardis Clinical and Genetic Laboratory, Mashhad, Iran.
Zoltan Lukacs - Metabolic Laboratory, Hamburg University of Medical Center, Hamburg, Germany.
Abdolreza Varasteh - Immunobiochemistry Lab, Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran - Varastegan Institute for Medical Sciences, Mashhad, Iran - Pardis Clinical and Genetic Laboratory, Mashhad, Iran.

Background: 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. Methods: Two hundred and fifty-one healthy subjects, including ۱۳۲ males and ۱۱۹ females, from ۲ days to ۱۵ years of age were enrolled. Urinary organic acids were extracted from urine with organic solvents and identified and quantified by gas chromatography-mass spectrometry. Results: The results provide a foundation on which to check results for patients with potentially abnormal organic acidurias. By this method ۹۸ organic acids were identified in a pediatric population of Iran. Conclusions: The present work identifies and quantifies major and minor urinary metabolites excreted by normal subjects. We also analyzed urine from ۳۰ patients with organic acid metabolism abnormalities and compared the concentrations of specific organic acids with those from urines of normal individuals.

Gas chromatography/mass spectrometry, Iran population, Normal individuals, Urine organic acid analysis, Urine organic acids range