MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.46
Current version: v.2.0
Updated: May 2014
Feedback/Comments :: View Release Notes
February 2013, v.1.46
Current version: v.2.0
Updated: May 2014
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
- 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.2.0, May 2014).
Resources
Overview
Some educational resources and tools are currently available to support nutrition management of MSUD. Development of additional resources specific to these guidelines is planned. A grant has been submitted by GMDI for funding the costs of developing a toolkit to accompany the guidelines.
Learning Objectives for Patients/Families
- Understand the basic defect of BCAA catabolism in MSUD
- Recognize the need to monitor clinical and biochemical signs of poor dietary control and metabolic decompensation
- Understand appropriate treatment during illness
- Recognize the need for frequent contact with the appropriate healthcare providers (metabolic dietitian, nurse, physician, etc)
- Acquire skills needed to implement the dietary prescription
- Realize the importance of strict adherence to the dietary prescription
- Recognize the role of medical food in the dietary management
Resources for patients
http://gmdi.org/index.php?option=com_content&view=article&id=110&Itemid=58
Resources for providers
http://gmdi.org/index.php?option=com_content&view=article&id=110&Itemid=58
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