Q. Long-term treatment of maple syrup urine disease?

Special feeds containing energy and proteins without the branched chain amino acids can be given to the infants. In some infants if the orogastric feeding is not acceptable, gastrostomy is initiated for initial care during the neonatal period. Long- term therapy for MSUD is by means of diet. Patients with classic MSUD are unable to terminate diet. Plasma concentration of isoleucine, leucine and valine should be maintained in the range of 40 to 90 m mol; 80 to 200 m mol y 200 to 425 m mol, respectively.

Long-term treatment of MSUD involves a carefully controlled diet which strictly limits dietary protein in order to prevent the accumulation of BCAAs in the blood. The major component of the diet is a special formula designed for children with MSUD. These MSUD formulas do not contain any leucine, isoleucine or valine but are otherwise nutritionally complete.