Which of the following is reduced in the diet in the treatment of maple syrup urine disease (MSUD)?

Question

Quizplus · Accepted Answer

MSUD is also known as branched-chain ketoaciduria, and this results from a defect in the branched-chain alpha-ketoacid dehydrogenase complex.The branched-chain amino acids-leucine, isoleucine, and valine-cannot be metabolized; therefore, affected persons require the use of specialty formulas that lack BCAAs.The BCAAs are slowly introduced into the diet when plasma leucine levels are decreased as they are necessary for growth to occur.However, dietary manipulation must occur so that enough BCAAs are available to support growth but not too high to increase plasma levels of the BCAAs.

Which of the Following Is Reduced in the Diet in the Treatment