Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0162275 (ketonuria)
 553 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 3-yr-old boy was investigated for numerous episodes of fatigue, irritability, pallor, and sweating, which began at 11 mo of age, when he had an episode of symptomatic hypoglycemia with ketonuria. He had euphoria, mental confusion, drowsiness, nausea, and vomiting 1-5 hr after oral administration of glycerol in doses of 0.5-1.0gm/kg. Orally administered MCT (1 gm/kg) had similar effects. On one occasion, oral glycerol also provoked hypoglycemia, as had a 16 1/2 hr fast. Intravenously administered glycerol (0.09 gm/kg) induced an immediate loss of consciousness from which he recovered spontaneously after 30 min; there were no changes in blood glucose values. Intravenously administered fructose (0.25 gm/kg) was tolerated normally. Leukocytes showed normal activities for FDPase, glycerol kinase, and glycerol phosphate dehydrogenase. The restriction of dietary intake of fat has been associated with a marked improvement in physical and mental activities. These observations suggest a unique, yet undifined intolerance to glycerol, which suggest caution in the diagnostic use of glycerol in the investigation of hypoglycemia as well as in the therapy of increased intracranial or intraocular pressure.
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PMID:Glycerol intolerance in a child with intermittent hypoglycemia. 16 54

In metabolically healthy volunteers we investigated the influence of long and medium chained triglycerides on the kinetic of the free fatty acids in blood and on protein metabolism. During a three hour infusion of lipid emulsion (infusion rate 120 mg/kg BW X h) consisting of 50% medium and 50% long chained triglycerides we observed a significant increase of the concentrations of the medium chained free fatty acids (caprylic and capric acid), which decreased down to basic values with a half life of 30 minutes immediately after the infusion period. The total concentration of free fatty acids was higher during the infusion of a MCT/LCT mixture than during the infusion of pure LCT. Glucose infused simultaneously reduced the metabolism of the free fatty acids. MCT induced higher ketonemia and ketonuria than LCT, the ketogenesis could be suppressed by additional glucose. Compared to the glycerol control infusion the total nitrogen excretion was reduced under lipid infusion. Beside the lower excretion of Glu + Gln and Ala the excretion of 3-methylhistidine was reduced, which indicates a decreased on muscle protein turnover as referred to increased free fatty acids and the ketone body concentrations respectively.
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PMID:[Effect of lipid infusions on triglyceride and protein metabolism]. 681 32