Dietary therapy must be lifelong. The intake of leucine is calculated on an individual basis following the measurement of plasma branched-chain amino acids. Measure plasma amino acid levels on a regular basis at appropriate intervals for the first 6-12 months of life. PO absorption is poor, but parenteral route may be associated with severe adverse reactions. Patients with MSUD are at risk for metabolic decompensation during periods of increased catabolism.
Dietary compliance is necessary to prevent developmental delay and neurological symptoms.
Educate patients and their caregivers about the principles of dietary treatment, calculation of leucine requirement, and intake and initial management of acute episodes. The Metabolic and Molecular Bases of Inherited Disease. Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. However, as medical science is constantly changing and human error is always possible , the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information.