Distinction of dicarboxylic aciduria due to medium-chain triglyceride feeding from that due to abnormal fatty acid oxidation and fasting in children.
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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Medical Research Service, Veterans Affairs Medical Center, Cleveland, OH 44106, USA.
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Kerr
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D002208:Caproates / Q000652:urine; D002210:Caprylates / Q000652:urine; D016022:Case-Control Studies; D003937:Diagnosis, Differential; D003998:Dicarboxylic Acids / Q000652:urine*; D005215:Fasting; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D006880:Hydroxy Acids; D007223:Infant; D007225:Infant Food / Q000009:adverse effects; D008661:Metabolism, Inborn Errors / Q000175:diagnosis / Q000378:metabolism*; D010084:Oxidation-Reduction; D014280:Triglycerides / Q000008:administration & dosage* / Q000009:adverse effects
D002208:Caproates; D002210:Caprylates; D003998:Dicarboxylic Acids; D005227:Fatty Acids; D006880:Hydroxy Acids; D014280:Triglycerides; C026717:7-hydroxyoctanoic acid; C016502:5-hydroxyhexanoic acid