Nutrition Management Guidelines for VLCAD | Southeast Regional Genetics Network

Management

Guidelines

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VLCAD Nutrition Management Guidelines

First Edition
February 2019, v.1.4
Updated: November 2022
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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. Nutrient intake
- 2. Illness Management
- 3. Nutrient supplementation and other treatments
- 4. Monitoring
- 5. Exercise
- 6. Pregnancy
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 VLCAD Phenotypes
- T.2 Laboratory and Clinical Findings in Severe VLCAD Deficiency
- T.3 Recommended Intakes of Essential Fatty Acids for Individuals with VLCAD
- T.4 Nutrition Problem Identification for VLCAD
- T.5 Medical Foods for the Nutrition Management of VLCAD
- T.6 Sources of Medium Chain Triglycerides (MCT)
- T.7 Recommendations for Fasting Intervals for Individuals with VLCAD when well
- T.8 Recommended Fat (total, long chain and medium chain), Energy and Protein Intakes for Individuals with VLCAD when Well
- T.9 Essential Fatty Acid Content of Selected Oils
- T.10 Monitoring the Nutritional Management of an Individual with VLCAD when Well
- T.11 Long-chain Fat Restriction and MCT Supplementation Recommendations for Individuals with VLCAD over the age of 1 year
- T.12 Long-chain Fat Restriction and MCT Supplementation Recommendations for Infants with VLCAD
- T.13 MCT Use in Individuals with VLCAD and Cardiomyopathy and/or Rhabdomyolysis
- T.14 European Consensus of Essential Fatty Acid Intakes
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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Monitoring and Evaluation

Biomarkers to Monitor

See (RECOMMENDATION TABLE #10, Monitoring the Nutritional Management of an Individual with VLCAD when Well) and nutrition recommendations for question 4.

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