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L-valine**
Maple syrup urine disease (MSUD)
With the help of metabolic dietitian: 20-120 mg/kg/day. Dose adjusted as necessary to achieve normal plasma aminoacids levels (4).
600 mg capsules
Powder
PO
Unknown
4
4. Winter SC. Treatment of carnitine deficiency. J Inherit Metab Dis 2003;26(2-3):171-80