Maple syrup urine disease-therapeutic use of insulin in catabolic states
- 1 November 1982
- journal article
- research article
- Published by Springer Nature in European Journal of Pediatrics
- Vol. 139 (3) , 172-175
- https://doi.org/10.1007/bf01377350
Abstract
High and neurotoxic blood levels of leucine and its ketoanalogue develop in catabolic patients with maple syrup urine disease. The use of relatively high doses of insulin and additional glucose had a more pronounced effect on lowering leucine (and α-ketoisocaproate) blood levels than dietary elimination of leucine alone. This is demonstrated in 2 neonates after blood exchange transfusion and in one 4-months old patient suffering from febrile diarrhea.Keywords
This publication has 26 references indexed in Scilit:
- Serum branched-chain amino acids in the diagnosis of hyperinsulinism in infancyThe Journal of Pediatrics, 1980
- Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acidsEuropean Journal of Pediatrics, 1980
- Insulin to Inhibit Protein Catabolism after InjuryNew England Journal of Medicine, 1979
- Correlations between branched-chain amino acids and branched-chain α-keto acids in blood in maple syrup urine diseaseClinica Chimica Acta; International Journal of Clinical Chemistry, 1978
- Blood amino acid levels in patients with insulin excess (functioning insulinoma) and insulin deficiency (diabetic ketosis)Metabolism, 1978
- Maple syrup urine disease: Treatment of the acutely ill newbornEuropean Journal of Pediatrics, 1978
- Amino Acid Metabolism in ManAnnual Review of Biochemistry, 1975
- Enzyme activity in classical and variant forms of maple syrup urine diseasePublished by Elsevier ,1972
- Treatment of Branched Chain Ketoaciduria (BCKA) During Acute IllnessClinical Pediatrics, 1972
- Accelerated chromatography of amino acids associated with phenylketonuria, leucinosis (maple syrup urine disease), and other inborn errors of metabolismAnalytical Biochemistry, 1967