MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.50
Current version: v.1.58
Updated: August 2016
Feedback/Comments :: View Release Notes
February 2013, v.1.50
Current version: v.1.58
Updated: August 2016
Feedback/Comments :: View Release Notes
- Overview
- Methodology
- Background
- Biochemical Pathway and Nutrition Treatment Rationale
- Nutrition Assessment
- Comparative Standards
- Nutrition Problem Identification
- Nutrition Intervention
- Nutrition Recommendations
- 1. Nutrition interventions during illness, trauma or surgery
- 2. Nutrition management and Branched chain amino acid (BCAA) blood concentrations
- 3. Thiamin supplementation
- 4. Nutrition interventions during pregnancy, at delivery and during the postpartum period
- 5. Nutrition interventions and liver transplantation
- Monitoring and Evaluation
- Resources
- Benefits and Harms of Implementing the Recommendations
- Barriers to Implementation
- Areas for Future Research
- List of Tables
-
Literature Evidence Summary Tables
- T.1 MSUD Phenotypes
- T.2 Laboratory and Clinical Findings for Classical MSUD
- T.3 Nutrient Recommended Intake and Sources in the Dietary Treatment of Well Individuals with MSUD
- T.4 Recommended Daily Nutrient Intakes of BCAA, PRO, ENERGY and Fluids for Infants, Children and Adults with MSUD (when well)
- T.5 Protein Recommendations during Pregnancy for MSUD
- T.6 Nutrition Problem Identification for MSUD based on the International Dietetics and Nutrition Terminology Reference Manual, 3rd Edition
- T.7 Recommendations for the Nutritional Monitoring of Individuals with MSUD
- T.8 Findings from Delphi Survey Round 1
- T.9 Findings from Delphi Survey Round 2
- T.10 Delphi Round 1 - Lab Test Frequency for Medically Stable Individuals
- T.11 Classification of Medical Foods for MSUD
- T.12 Recommended Nutrient Intake for the School-aged Child with MSUD
- T.13 Sample Menu for Case 5.1: school age child with MSUD
MSUD Toolkit Tables- T.14 Recommended Nutrient Intake for Newborn Infant with MSUD
- T.15 Medical Food and Breast Milk Composition for Case 3.1
- T.16 Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using breast milk
- T.17 Rounded Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using breast milk
- T.18 Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using infant formula
- T.19 Recommended Nutrient Intake for Older Infants with MSUD
- T.20 Calculation of Current Nutrient Intake for Case 4.1
- T.21 Introduction of Solids for Case 4.1
- T.22 Sample Menu with Suggested Feeding Schedule for Case 4.1: infant with MSUD starting solids
- T.23 Nutrition Goals During Acute Illness
- T.24 Monitoring During Acute Illness
- References
- Contributors
- Appendix A: Recommendation Rating Definitions
- Appendix B: Terms
- Disclaimer
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This version is not current. Click to view the most recent edition (v.1.58, August 2016).
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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