October 2024, v.2.0
Updated: October 2024
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- 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
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
- Contributors
- Appendix A: Recommendation Rating Definitions
- Appendix B: Terms
- Disclaimer
The Nutrition Management Guideline for Individuals with Maple Syrup Urine Disease (MSUD) is part of a larger project undertaken by the Southeast Newborn Screening and Genetics Collaborative (SERC) (HRSA Region 3) and Genetic and Metabolic Dietitians International (GMDI) to develop nutrition management guidelines for inherited metabolic disorders (IMD).
The purpose of this guideline is to foster optimum nutrition management of individuals with MSUD and reduce the uncertainty and variability in the management of this IMD.
The target population includes individuals with MSUD at all life stages from the acute newborn period, through childhood and adolescence, to ongoing management in the adult years and during pregnancy. These guidelines include management during periods of health and illness.
The guidelines are intended for use by metabolic dietitians, physicians, others of the health care team and researchers who collaborate to provide care and advice for individuals with MSUD and their families, advocates, and others.
Sponsorship is shared by the Southeast Regional Newborn Screening and Genetics Collaborative (SERC) and Genetic Metabolic Dietitians International (GMDI).
Funding for this project has been provided by Health Resources and Services Administration (HRSA) grant number: #H46MC24090.
- Describes the methodology used for development of evidence and consensus-based guidelines
- Provides background about MSUD, its diagnosis, biochemical pathway and nutrition treatment rationale
- Describes nutrition care in MSUD, including assessment, nutrition problem identification, nutrition intervention with evidence- and consensus-based recommendations, and monitoring and evaluation
- Lists specific recommendations with grades
- Explores implementation considerations and needed research