Abstract
Summary: We present case histories of two young children with episodes of hypoglycemia, elevation of SGOT, low insulin levels, increased urinary excretion of Ψ-hydroxy fatty acids (5-hydroxyhexanoic, 7-hydroxyoctanoic and 9-hydroxydecanoic), traces of the corresponding Ψ-ketoacids and elevations of urinary adipic, suberic, and sebacic acids. The ratio of Ψ-hydroxy fatty acids to 3-hydroxybutyric in the urine of these patients is higher than in patients of similar ages with similar illnesses. These acids persisted while the patients were well. Increased urinary Ψ-hydroxy fatty acids could be reproduced by a load of medium chain triglycerides without precipitating other clinical symptoms. Three children with hypoglycemia were found not to excrete measurable amounts of these unusual acids while ill. A medium chain triglyceride load in one of these children after recovery failed to elicit Ψ-hydroxy acid excretion. Small amounts of urinary 5-hydroxyhexanoic acid only were found in two patients with acute Reye's syndrome and in three of five severely ill children with starvation ketonuria. In this last group, no urinary Ψ-hydroxyacids could be detected after recovery. Normal children do not excrete measurable amounts (less than 1 mg/g creatinine) of these Ψ-hydroxyacids.
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Colle, E., Mamer, O., Montgomery, J. et al. Episodic Hypoglycemia with Ψ-Hydroxy Fatty Acid Excretion. Pediatr Res 17, 171–176 (1983). https://doi.org/10.1203/00006450-198302000-00018
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DOI: https://doi.org/10.1203/00006450-198302000-00018
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