Management
Guidelines
Portal
MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.50
Current version: v.1.58
Updated: August 2016
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Areas for Future Research
Areas for Future Research

The guidelines for nutritional management are based on the best available evidence as well as survey results and expert opinion. In nearly all areas of these management and monitoring guidelines, collection of more clinical data, well-designed clinical trials, and use of patient registries have the potential to increase the validity of the recommendations. In addition, future research (Y.4) will be able to evaluate experimental therapies, that are now being investigated, such as: 

  • Regulation of MSUD BCAA levels with phenylbutyrate (L.5, Y.2, Y.3)
  • Use of antioxidants (L.4, Y.6)
  • Treatment with norleucine ( L.7, Y.7)
  • Hepatocyte transplantation (L.6)

 

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