Distinction of dicarboxylic aciduria due to medium-chain triglyceride feeding from that due to abnormal fatty acid oxidation and fasting in children

Metabolism: Clinical and Experimental
K Y TserngD S Kerr

Abstract

Increased amounts of dicarboxylic acids are excreted in human urine under conditions of medium-chain triglyceride (MCT) feeding, abnormal fatty acid oxidation (FAO) and fasting. Criteria to distinguish dicarboxylic aciduria originating from MCT feeding and other conditions are needed in urinary organic acid profiling for detecting inborn errors of metabolism. Patterns of dicarboxylic aciduria in children under various conditions were compared. The relative amounts of medium-chain saturated dicarboxylic acids in urine are not reliable for identifying MCT-induced dicarboxylic aciduria. On the other hand, low ratios of unsaturated to saturated dicarboxylic acids (<0.1) and 3- hydroxydecenedioic to 3-hydroxydecanedioic acids were found to be useful in identifying dicarboxylic aciduria due to MCT ingestion. Additional unique features of dicarboxylic aciduria from MCT are low ratios of 3-hydroxydodecanedioic to 3-hydroxydecanedioic acid (<0.14) and 3-hydroxyadipic to adipic acid (<0.02).

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Citations

Apr 27, 1999·Clinica Chimica Acta; International Journal of Clinical Chemistry·J L PotterW G Kofron
Dec 17, 2002·Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences·Daniel JardinesJesús Molerio
May 14, 2016·Infection, Genetics and Evolution : Journal of Molecular Epidemiology and Evolutionary Genetics in Infectious Diseases·Zhang LinWeidong Zhang
May 8, 2016·Nutrition in Clinical Practice : Official Publication of the American Society for Parenteral and Enteral Nutrition·Maria AndersonJames Shoemaker
Jan 3, 2006·JPEN. Journal of Parenteral and Enteral Nutrition·Alison L SteiberLorraine J Weatherspoon
Oct 7, 2005·Nutrition in Clinical Practice : Official Publication of the American Society for Parenteral and Enteral Nutrition·Brian Schreiber
May 16, 2013·The Journal of Biological Chemistry·Jay S KirkwoodJan F Stevens

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