February 2019, v.1.1
Current version: v.1.4
Updated: June 2021
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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
Future Research Questions
What are the outcomes of patients treated with diets containing various percentages of long chain fat and medium chain fat?
Are outcomes in severe VLCAD improved if the diet contains less than 10% of energy as long chain fat?
Does a diet high in protein improve outcomes?
Do patients treated with triheptanoin have improved outcomes as compared to those treated with MCT?
Is L-carnitine supplementation safe during illness?
Are there benefits of mitochondrial-enhancing supplements in patients with VLCAD and cardiomyopathy?
What is the pathophysiology of rhabdomyolysis and cardiomyopathy in VLCAD?
What are best laboratory measurements to assess effectiveness of diet treatment?
Does a plasma acylcarnitine profile provide useful information to assess effectiveness of the diet and/or suggest need for changes in diet treatment?
What are pregnancy outcomes in VLCAD?
Is muscle MRI useful in understanding the pathophysiology of myopathy in VLCAD( F.4235"> F.4235 )?
Does the method of skimming breast milk (centrifuge vs non-centrifugal separation) affect the fat content of breast milk and the appropriateness for use in feeding infants with VLCAD?
Does genotype affect phenotype and nutrition recommendations, and if so, how?