The diet of a MSUD patient (child) should therefore involve:

• Measured quantities of natural protein or leucine from foods.

• A BCAA free protein, vitamin and mineral supplement.

• The natural protein and vitamin/mineral supplement should ideally be evenly distributed through the day to allow maximum utilization of the amino acids for protein anabolism, and for tolerance of the supplement.

• Free foods that are low in BCAA including sugars, fats and oils, as well as, foods specifically produced for a low protein diet (breads, biscuits, pastas elc.) And supplements be given.

• Supplements of valine and/or isoleucine are helpful.