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MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.55
Current version:
v.2.0
Updated: June 2021
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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
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
MSUD Toolkit Tables
T.8
Recommended Nutrient Intake for Newborn Infant with MSUD
T.9
Medical Food and Breast Milk Composition for Case 3.1
T.10
Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using breast milk
T.11
Rounded Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using breast milk
T.12
Calculation of Formula Mix to Meet Recommended Intake for Case 3.1: using infant formula
T.13
Recommended Nutrient Intake for Older Infants with MSUD
T.14
Calculation of Current Nutrient Intake for Case 4.1
T.15
Introduction of Solids for Case 4.1
T.16
Sample Menu with Suggested Feeding Schedule for Case 4.1: infant with MSUD starting solids
T.17
Recommended Nutrient Intake for the School-aged Child with MSUD
T.18
Sample Menu for Case 5.1: school age 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.2.0, June 2021).
Appendix B: Terms
Important Terms Used in This Document
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