Nutrition Management Guidelines for VLCAD | Southeast Regional Genetics Network

Management

Guidelines

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VLCAD Nutrition Management Guidelines

First Edition
February 2019, v.1.1
Current version: v.1.4
Updated: June 2021
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. 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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This version is not current. Click to view the most recent edition (v.1.4, June 2021).

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. These include population-based growth charts, nutrient intake recommendations and laboratory norms, as well as disorder-specific guidelines.

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Comparison of nutrient intake to established standards

For comparison of nutrient intake to established standards of the Dietary Reference Intakes (DRI) see: http://fnic.nal.usda.gov/dietary-guidance/dietary-reference-intakes/dri-tables

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