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Eur J Pediatr. 1982 Nov;139(3):172-5.

Maple syrup urine disease--therapeutic use of insulin in catabolic states.

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 alpha-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.

PMID:
6761134
DOI:
10.1007/bf01377350
[Indexed for MEDLINE]

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