Nutrition Management Guidelines for MSUD | Southeast Regional Genetics Network

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Management

Guidelines

Portal

DNDF Model

MSUD Nutrition Management Guidelines

First Edition
February 2013, v.1.54
Current version: v.1.58
Updated: April 2018
Feedback/Comments :: View Release Notes

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 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
- 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.1.58, April 2018).

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.

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Comparison of nutrient intake to established standards

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