Episodic hypoglycemia with psi-hydroxy fatty acid excretion.
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.
D001219:Aspartate Aminotransferases / Q000097:blood; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006880:Hydroxy Acids / Q000652:urine*; D007003:Hypoglycemia / Q000175:diagnosis* / Q000378:metabolism; D007005:Hypoglycins / Q000506:poisoning; D007223:Infant; D007328:Insulin / Q000097:blood / Q000172:deficiency; D007651:Keto Acids / Q000652:urine; D010939:Plant Poisoning / Q000175:diagnosis; D012202:Reye Syndrome / Q000175:diagnosis
D006880:Hydroxy Acids; D007005:Hypoglycins; D007328:Insulin; D007651:Keto Acids; D001219:Aspartate Aminotransferases