Episodic hypoglycemia with psi-hydroxy fatty acid excretion.

Abstract

We present case histories of two young children with episodes of hypoglycemia, elevation of SGOT, low insulin levels, increased urinary excretion of psi-hydroxy fatty acids (5-hydroxyhexanoic, 7-hydroxyoctanoic and 9-hydroxydecanoic), traces of the corresponding psi-ketoacids and elevations of urinary adipic, suberic, and sebacic acids. The ratio of psi-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 psi-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 psi-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 psi-hydroxyacids could be detected after recovery. Normal children do not excrete measurable amounts (less than 1 mg/g creatinine) of these psi-hydroxyacids.

• Authors: E Colle, O A Mamer, J A Montgomery, J D Miller
• Journal: Pediatric research (Pediatr Res) Vol. 17 Issue 2 Pg. 171-6 (Feb 1983) ISSN: 0031-3998 [Print] United States
• PMID: 6402754 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Hydroxy Acids
• Hypoglycins
• Insulin
• Keto Acids
• Aspartate Aminotransferases

Topics

• Aspartate Aminotransferases (blood)
• Diagnosis, Differential
• Female
• Humans
• Hydroxy Acids (urine)
• Hypoglycemia (diagnosis, metabolism)
• Hypoglycins (poisoning)
• Infant
• Insulin (blood, deficiency)
• Keto Acids (urine)
• Plant Poisoning (diagnosis)
• Reye Syndrome (diagnosis)