Episodic Hypoglycemia with Ψ-Hydroxy Fatty Acid Excretion View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

1983-02

AUTHORS

Eleanor Colle, J A Montgomery, John D Miller, O A Mamer

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. More... »

PAGES

171

References to SciGraph publications

Identifiers

URI

http://scigraph.springernature.com/pub.10.1203/00006450-198302000-00018

DOI

http://dx.doi.org/10.1203/00006450-198302000-00018

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1017722926

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/6402754


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