MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.54
Current version: v.2.0
Updated: April 2018
Feedback/Comments :: View Release Notes
February 2013, v.1.54
Current version: v.2.0
Updated: April 2018
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 Dietary PRO, BCAA and Energy Intake
- T.5 Nutrition Problem Identification for MSUD based on the International Dietetics and Nutrition Terminology Reference Manual, 3rd Edition
- T.6 Recommendations for the Nutritional Monitoring of Individuals with MSUD
- T.7 Classification of Medical Foods for MSUD
- T.8 Sample Menu for Case 5.1: school age child with MSUD
MSUD Toolkit Tables- T.9 Recommended Nutrient Intake for Newborn Infant with MSUD
- T.10 Medical Food and Breast Milk Composition for Case 3.1
- T.11 Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using breast milk
- T.12 Rounded Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using breast milk
- T.13 Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using infant formula
- T.14 Recommended Nutrient Intake for Older Infants with MSUD
- T.15 Calculation of Current Nutrient Intake for Case 4.1
- T.16 Introduction of Solids for Case 4.1
- T.17 Sample Menu with Suggested Feeding Schedule for Case 4.1: infant with MSUD starting solids
- T.18 Recommended Nutrient Intake for the School-aged Child with MSUD
- T.19 Nutrition Goals During Acute Illness
- T.20 Monitoring During Acute Illness
- T.21 Recommended Nutrient Intake for Pregnant Woman with MSUD
- T.22 Recommended Nutrient Intakes for an Adult Woman with Classical MSUD (Non-pregnant)
- T.23 24-Hour Dietary Intake of an Adult Woman with Classical MSUD (non-pregnant)
- T.24 Calculation of Current Nutrient Intake for AC Prior to Pregnancy
- T.25 24-Hour Intake Modified for Increased Intact Protein
- T.26 Calculation of Current Nutrient Intake for AC at 30 Weeks Gestation
- References
- Contributors
- Appendix A: Recommendation Rating Definitions
- Appendix B: Terms
- Disclaimer
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Comparative Standards
Overview
Comparative standards are reference values from reputable and authoritative sources such as government agencies, independent scientific panels, standards organizations, and scientific and professional organizations. See TABLE #4, Recommended Dietary PRO, BCAA and Energy Intake for more information.
Comparison of nutrient intake to established standards
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