Nutrition Management Guidelines for MSUD | Southeast Regional Genetics Network

Management

Guidelines

Portal

MSUD Nutrition Management Guidelines

First Edition
February 2013, v.1.50
Current version: v.2.0
Updated: August 2016
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Overview
- Nutrition Guidelines Project
- Purpose
- Target Population
- Intended Users of the Guidelines
- Sponsor Organization
- Funding
- Content Highlights
Methodology
- Overview
- Question Formulation
- Search Process
- Critical Appraisal and Abstraction
- Evidence Summary
- Development of Recommendations
- Portal Application
- Plan for Updating
Background
- Introduction to Guidelines
- Newborn Screening
- Confirmatory Testing
- Genetics
Biochemical Pathway and Nutrition Treatment Rationale
- Biochemical Pathway Figure
- Nutrition Treatment Rationale
Nutrition Assessment
- Patient History
- Anthropometrics
- Nutrition-focused Physical Findings
- Laboratory Findings, Medical Tests and Procedures
- Food and Nutrition-related History
Comparative Standards
- Overview
- Comparison of nutrient intake to established standards
Nutrition Problem Identification
- Overview
- Common Nutrition Diagnoses
Nutrition Intervention
- Overview
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
- Biomarkers to Monitor
Resources
- Overview
- Learning Objectives for Patients/Families
- Resources for patients
- Resources for providers
Benefits and Harms of Implementing the Recommendations
- Overview
- Potential Harms
- Potential Benefits
Barriers to Implementation
- Overview
- Infrastructure
- Patient-related
- Regulatory
Areas for Future Research
- Areas for Future Research
List of 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
- Analyzed Literature
- Reference Materials
Contributors
- Key Project Personnel
Appendix A: Recommendation Rating Definitions
- Strength of Recommendation Rating
- Clinical Action/Application
Appendix B: Terms
- Important Terms Used in This Document
Disclaimer
- Disclaimer

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This version is not current. Click to view the most recent edition (v.2.0, August 2016).

Appendix B: Terms

Important Terms Used in This Document

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