DISTINCTION OF DICARBOXYLIC ACIDURIA DUE TO MEDIUM-CHAIN TRIGLYCERIDEFEEDING FROM THAT DUE TO ABNORMAL FATTY-ACID OXIDATION AND FASTING INCHILDREN

Increased amounts of dicarboxylic acids are excreted in human urine un der conditions of medium chain triglyceride (MCT) feeding, abnormal fa tty acid oxidation (FAO) and fasting, Criteria to distinguish dicarbox ylic aciduria originating from MCT feeding and other conditions are ne eded in urinary organic acid profiling for detecting inborn errors of metabolism, Patterns of dicarboxylic aciduria in children under variou s conditions were compared. The relative amounts of medium chain satur ated dicarboxylic acids in urine are not reliable for identifying MCT- induced dicarboxylic aciduria. On the other hand, low ratios of unsatu rated to saturated dicarboxylic acids (<0.1) and 3-hydroxydecenedioic to 3-hydroxydecanedioic acids were found to be useful in identifying d icarboxylic aciduria due to MCT ingestion. Additional unique features of dicarboxylic aciduria from MCT are low ratios of 3-hydroxydodecaned ioic to 3-hydroxydecanedioic acid (<0.14) and 3-hydroxyadipic to adipi c acid (<0.02). (C) 1996 by W.B. Saunders Company