February 2019, v.1.0
Current version: v.1.4
Updated: February 2019
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- Overview
- Methodology
- Background
- Biochemical Pathway and Nutrition Treatment Rationale
- Nutrition Assessment
- Comparative Standards
- Nutrition Problem Identification
- Nutrition Intervention
- Nutrition Recommendations
- 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 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
- Contributors
- Appendix A: Recommendation Rating Definitions
- Appendix B: Terms
- Disclaimer
The Nutrition Management Guideline for individuals with very-long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is part of a larger project undertaken by the Southeast Regional Genetics Network (SERN) (HRSA Region 3) and Genetic 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 VLCAD and reduce the uncertainty and variability in the management of this IMD.
The target population includes individuals with VLCAD at all life stages from the newborn period, through childhood and adolescence, to 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 VLCAD as well as their families, advocates, and others.
Sponsorship is shared by SERN and GMDI.
Funding for this project has been provided by Health Resources and Services Administration (HRSA) grant number: #UH7MC30772 and GMDI.
- Methodology used for development of evidence and consensus-based guidelines
- Background information about VLCAD, its diagnosis, biochemical pathway and nutrition treatment rationale
- Nutrition care in VLCAD, including assessment, nutrition problem identification, nutrition intervention with evidence- and consensus-based recommendations, and monitoring and evaluation
- Specific recommendations with ratings based on the strength of the evidence
- Consideration for implementation
- Direction for future research