Abstract
Two female children with febrile episodic hypoglycemia(glucose 10 and 34 mgm/dl) and elevation of SGOT (103 and 163 U/L) had increased amounts of urinary C6-10 dicarboxylic fatty acids as well as the ω-1-OH and ketoderivatives of these acids. Aceto-acetate and β-OH butyric acid are only moderately elevated.
The compounds are present between attacks, increase after a medium (MCT) or long (LCT) chain triglyceride load and are not found in the urine of normal children. Young infants with ketosis will show dicarboxylic aciduria but only in the presence of greatly increased acetoacetate, β-OH butyric and lactic acid.
The data suggest a deficiency of medium chain acyl coA dehydrogenase activity limiting β-oxidation of fatty acids which becomes significant during periods of increased energy demands.
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Miller, J., Colle, E., Mamer, O. et al. 1165 EPISODIC HYPOGLYCEMIA WITH ORGANIC ACIDURIA. Pediatr Res 15 (Suppl 4), 637 (1981). https://doi.org/10.1203/00006450-198104001-01191
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DOI: https://doi.org/10.1203/00006450-198104001-01191