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Maple syrup urine disease-therapeutic use of insulin in catabolic states (1982)

Wendel, U. ; Langenbeck, U. ; Lombeck, Ingrid ; [et al.]

Springer

European journal of pediatrics 139 (1982), S. 172-175

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ISSN: 1432-1076

Keywords: Maple syrup urine disease ; Acute phase of MSUD ; Insulin treatment

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: 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.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/BF01377350

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