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
Phenotype | Newborn screen | Diagnosis | Clinical Symptoms | Biochemical |
Mild | Mild to moderately elevated concentrations of long chain acylcarnitines | Diagnostic testing confirms the diagnosis, although plasma acylcarnitine profile may be near normal | -Asymptomatic beyond infancy -Tolerates catabolic stressors without metabolic decompensation -May present with rhabdomyolysis, muscle pain and/or exercise intolerance during adolescence and adulthood | Often normal for healthy individuals |
Moderate | Mild to moderately elevated concentrations of long chain acylcarnitines | Diagnostic testing includes an abnormal plasma acylcarnitine profile | -Asymptomatic at diagnosis -Episodic hypoketotic hypoglycemia or rhabdomyolysis precipitated by catabolic illness, fasting or exercise -Cardiomyopathy or other cardiac presentation unlikely. | Abnormal even when healthy |
Severe | Significantly elevated concentrations of long chain acylcarnitines. | Diagnostic testing includes an abnormal plasma acylcarnitine profile | -Symptomatic at diagnosis or within first months of life without treatment -May include hypertrophic or dilated cardiomyopathy, pericardial effusion and arrhythmias, as well as hypotonia, hepatomegaly, intermittent hypoglycemia and rhabdomyolysis. | Abnormal even when healthy |
Findings | Symptomatic | Treated In Metabolic Control | During Illness |
Laboratory | |||
Plasma acylcarnitines1 | C14:1 >1 mmol/L2 | ↑ | ↑↑ |
Plasma carnitine | nl or ↓ | nl | nl or ↓ |
Glucose | Intermittent, hypoketotic hypoglycemia3 | nl | nl or hypoketotic hypoglycemia |
Ketones (blood/urine) | ↓ or absent3 | nl or ↓ | ↓ or absent |
Creatine kinase | ↑ or nl | ↑ or nl | ↑ |
Urine organic acids | C6-C14 dicarboxylic acids present | C6-C14 dicarboxylic acids present | C6-C14 dicarboxylic acids present |
Liver function tests | ↑ or nl | ↑ or nl | ↑ or nl |
Metabolic/lactic acidosis | + | - | + |
Clinical | |||
Poor Feeding | +/- | - | + |
Nausea/vomiting | +/- | - | + |
Lethargy | +/- | - | + |
Seizures | +/- | - | +/- |
Coma | +/- | - | +/- |
Cardiac: hypertrophic cardiomyopathy4, pericardial effusion, cardiac decompensation, arrhythmias | +/- | +/- | +/- |
Hepatomegaly5 | +/- | - | +/- |
Encephalopathy | +/- | - | +/- |
Rhabdomyolysis | +/- | - | +/- |
Exercise Intolerance | NA | +/- | NA |
Hypotonia | +/- | - | +/- |
SIDS | Possible | NA | NA |
1C14:2, C14, C12:1 are also elevated
2Cut-off values for elevations of C14:1 and other long-chain metabolites can vary among newborn screening programs
3Hypoketotic hypoglycemia is a hallmark finding in long chain fatty acid oxidation disorders
4Cardiomyopathy may persist while on dietary therapy
5Hepatomegaly is due to fatty infiltration of liver
Intake Recommendations for Essential Fatty Acids | |||||
Institute of Medicine: Adequate Intake (AI)1 | |||||
Age | LA2 | ALA | DHA | DHA+EPA | ARA |
g/d | mg/d | Not provided | |||
0 - 6 mo | 4.4 | 500 | - | - | - |
7 - 12 mo | 4.6 | 500 | - | - | - |
1 - 3 yr | 7 | 700 | - | - | - |
4 - 8 yr | 10 | 900 | - | - | - |
9 - 13 yr Males Females | 12 10 | 1200 1000 | - | - | - |
14 - 18 yr Males Females | 16 11 | 1600 1100 | - | - | - |
19 - 50 yr Males Females | 17 12 | 1600 1100 | - | - | - |
Pregnancy | 13 | 1400 | - | - | - |
Lactation | 13 | 1300 | - | - | - |
Food and Agriculture Organization/World Health Organization (FAO/WHO): Adequate Intake (AI)3 | |||||
Age | LA2 | ALA | DHA | DHA+EPA | ARA |
% Energy4 | % Energy | % Energy | mg/d | % Energy | |
0-6 mo | - | 0.2 - 0.3 | 0.1 - 0.18 | - | 0.2 - 0.3 |
Age | LA2 | ALA | DHA | DHA+EPA | ARA |
% Energy4 | % Energy | mg/kg | mg/d | - | |
6-24 mo | 3 - 4.5 | 0.4 - 0.6 | 10 - 12 | - | - |
2 - 4 yr | ≥ 2.5 | ≥ 0.5 | - | 100 - 150 | - |
4 - 6 yr | ≥ 2.5 | ≥ 0.5 | - | 150 - 200 | - |
6 - 10 yr | ≥ 2.5 | ≥ 0.5 | - | 200 - 250 | - |
Age | LA2 | ALA | DHA | DHA+EPA | ARA |
% Energy4 | % Energy | mg/d | mg/d | - | |
Pregnancy | ≥ 2.5 | ≥ 0.5 | 200 | 300 | - |
Lactation | ≥ 2.5 | ≥ 0.5 | 200 | 300 | - |
European Society for Pediatric Gastroenterology and Nutrition (ESPGHAN)5 | |||||
Age | LA2 | ALA | DHA | DHA+EPA | ARA |
mg/kg | mg/kg | mg/kg | mg/kg | mg/kg | |
Premature Infants6 | 385 - 1540 | ≥ 55 | 12 - 30 | - | 18 - 42 |
1Data summarized from Dietary Reference Intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein and amino acids, 2005.
2Abbreviations for fatty acids: LA: linoleic acid; ALA: alpha linolenic acid; DHA: docosahexaenoic acid; EPA: eicosopentanoic acid; ARA: arachidonic acid
3Data summarized from Fats and fatty acids in human nutrition: Report of an expert panel, 2010 (L.342).
4Intakes expressed as percent of total energy
5Data summarized from Committee on Nutrition, ESPGHAN, 2010 (L.345).
6Birth weight defined as 1000-1800 g
Nutrition Diagnosis1 (Problem) | Related to (Etiology) | As Evidenced By (Signs and Symptoms) |
Based on assessment findings, specify the current nutrition-related problem(s) to be addressed through nutrition management. | Identify the most pertinent underlying cause(s) or contributing risk factors for the specific problem. The etiology is commonly the target of nutrition intervention. | List subjective and objective data that characterize the specific problem and are also used to monitor nutrition intervention and outcomes |
Examples of specific nutrition problems: | Examples of underlying causes of the problem: | Examples of data used to determine and monitor the problem: |
Intake Domain Predicted excessive energy intake Predicted suboptimal energy intake Excessive fat intake Inadequate fat intake Predicted inadequate nutrient intake (specify e.g. MCT) Intake of type of fat inconsistent with needs (specify e.g., excessive LCFA, inadequate MCT) Inadequate essential fatty acid intake Excessive enteral nutrition infusion Inadequate enteral nutrition infusion Enteral nutrition composition inconsistent with needs Excessive duration between feeds (fasting) Clinical Domain Impaired nutrient utilization Altered nutrition-related lab values Food-medication interaction (specify) Growth rate below expected Underweight Overweight/obesity Behavioral-Environmental Domain Food and nutrition-related knowledge deficit Limited adherence to nutrition-related recommendations Limited access to food Limited access to medical food | Consumption Factors Lack of medical food2 consumption Suboptimal medical food2 consumption Excessive intake of (specify food or beverage) Inadequate intake of (specify food or beverage) Long duration between feedings Provider Factors Nutrition prescription no longer meets fat intake needs Nutrition prescription no longer meets energy needs Underlying Disease Factors New diagnosis of VLCAD Onset of cardiomyopathy or rhabdomyolysis Acute illness or infection Poor appetite due to (specify) Patient/Caretaker Knowledge and Behavior Factors Food choices suboptimal Lack of knowledge Limited adherence to dietary therapy recommendations Presentation to clinic for initial nutrition education Presentation to clinic for revised nutrition education (specify, e.g., illness, growth, pregnancy) Change in nutrient needs (specify, e.g MCT, energy) due to new or increased physical activity Access Factors Lack of financial resources for medical food2 Lack of medical insurance Inadequate third-party or denial of coverage for medical foods2 Lack of access to resources for care | From Biochemical Tests Laboratory value compared to norm or goal (specify) (e.g. CK, acylcarnitine) From Anthropometrics Growth pattern, weight, weight-for-height or BMI compared to standards (specify) Weight gain/loss (specify weight change) over the past (specify time frame) From Clinical/Medical Exam or History New diagnosis of VLCAD Micronutrient deficiency (physical sign or lab value) EFA deficiency (lab value or physical sign) From Diet History Estimated or calculated intake from diet record or dietary recall, compared to recommendation or individual's nutrition prescription (specify) Incomplete/inadequate/missing diet history or recall From Patient Report Verbalized lack of skill or understanding to implement nutrition recommendations Lack of appreciation for the importance of making nutrition-related changes Lack of social or familial support |
1Table content is based on Nutrition Care Process (NCP) terminology developed by the Academy of Nutrition and Dietetics (eNCPT. 2018 edition). NCP uses the following structure for documenting nutrition problems: nutrition diagnosis (Problem), related to (Etiology), and as evidenced by (Signs and Symptoms). Examples listed identify concerns particular to VLCAD and are grouped in domains of: Intake, Clinical, and Behavioral-Environmental. Problems identified may relate to any Etiology and be evidenced by any Signs and Symptoms within a domain.
2Low long-chain fat, high MCT medical food (formula). Refer to RECOMMENDATION TABLE #5, Medical Foods for the Nutrition Management of VLCAD for types of medical foods.
Medical Food1 | Quantity | Energy (kcal) | Prot | CHO | Total Fat (g) | LCT (g) | MCT (g) | LA | ALA |
Pregestimil2 | 100 g | 500 | 14 | 51 | 28 (48) | 12.7 (23) | 15.3 (26) | 4700 | 590 |
Lipistart3 | 100 g | 450 | 13.7 | 55 | 20.6 (38) | 3.8 (8) | 16.5 (30) | 1730 | 240 |
Monogen4 | 100 g | 444 | 12.9 | 69 | 12.9 (24) | 2.1 (4) | 10.8 (20) | 900 | 170 |
Enfaport5 | 100 ml | 103 | 3.6 | 10 | 5.6 (46) | 1.0 (9) | 4.6 (37) | 420 | 60 |
Source: MetabolcPro© (http://metabolicpro.org), Accessed 10/11/18
Some values have been rounded to nearest whole number or to one decimal.
1Examples of products available in the U.S. as of October 2018. Inclusion in table does not represent endorsement.
2 Mead Johnson Nutrition, Evansville, IN
3Vitaflo USA, Alexandria, VA
4Nutricia North America, Gaithersburg, MD
5Mead Johnson Nutrition, Evansville, IN; (ready to feed formula with concentration of 30 kcal/oz)
Product1 | Quantity | Energy (kcal) | Total Fat | MCT | Comments |
betaquik2 | 1 ml | 1.9 | 0.21 | 0.20 | Packaging: 225 ml bottle |
Liguigen3 | 1 ml | 4.5 | 0.5 | 0.45 | Packaging: 250 ml bottle |
MCT Oil4 | 1 ml | 7.7 | 0.93 | 0.93 | Packaging: 1 quart (946 ml) glass bottle |
MCT Procal2 | 1 g | 6.6 | 0.63 | 0.62 | Packaging: 1 sachet = 16 g |
MCT Procal2 | 1 sachet | 105.1 | 10.1 | 9.95 | 1 sachet = 2 scoops |
Source: MetabolcPro© (https://metabolicpro.org), Accessed 10/23/18
1Examples of products available in the U.S. as of October 2018. Inclusion in table does not represent endorsement.
2Vitaflo USA, Alexandria, VA
3Nutricia North America, Gaithersburg, MD
4Nestlé Health Science, Bridgewater, NJ
Age | Disease Severity | Maximum duration between feedings (hours) |
0 to < 4 mos | Severe1 | 3 |
Moderate | 3 to 4 | |
Mild | ||
4 to < 6 mos | Severe1 | 4 |
Moderate | 4 to 6 | |
Mild | ||
6 to < 9 mos | Severe1 | 6 |
Moderate | 6 to 8 | |
Mild | ||
9 to < 12 mos | Severe1 | 8 |
Moderate | 8 to 10 | |
Mild | ||
> 12 mos | Severe1 | 10 |
Moderate | 10 to 12 | |
Mild |
1 Based on clinical signs and symptoms, some individuals with severe VLCAD may require shorter intervals between feedings.
| Age | Disease Severity | Total Fat (% of total energy)1 | Long-Chain Fat (% of total energy) | Medium-Chain Fat (% of total energy) | Energy2 | Protein5 (g/kg/d) |
| 0-6 mos | Severe | 40-55 | 10-15 | 30-45 | EER | ≥1.5 |
| Moderate | 15-30 | 10-30 | ||||
| Mild | 30-55 | 0-20 | ||||
| 7-12 mos | Severe | 35-45 | 10-15 | 25-30 | EER | ≥1.2 |
| Moderate | 15-30 | 10-25 | ||||
| Mild | 30-40 | 0-10 | ||||
| 1-3 years | Severe | 30-40 | 10-15 | 10-30 | EER | ≥1.1 |
| Moderate | 20-30 | 10-20 | ||||
| Mild | 20-40 | 0-10 | ||||
| 4-18 years | Severe | 25-35 | 10 | 15-25 | EER with PAL3 | 0.85-0.95 |
| Moderate | 15-25 | 10-20 | ||||
| Mild | 20-35 | 0-10 | ||||
| >19 years | Severe | 20-35 | 10 | 10-25 | EER with PAL3 | 0.8 |
| Moderate | 15-20 | 10-20 | ||||
| Mild | 20-35 | 0-10 | ||||
| Pregnancy | Severe | 20-35 | 10 | 10-25 | EER per trimester4 | 1.1 |
| Moderate | 15-20 | 10-20 | ||||
| Mild | 20-35 | 0-10 | ||||
| Lactation | Severe | 20-35 | 10 | 10-25 | EER | 1.3 |
| Moderate | 15-25 | 10-20 | ||||
| Mild | 20-35 | 0-10 |
1 Total fat recommendations based on Adequate Intakes (AI) established by the Institute of Medicine (Dietary Reference Intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein and amino acids, 2005) and AI established by the Food and Agriculture Organization of the United Nations (FAO) (Fats and fatty acids in human nutrition: Report of an expert consultation, 2010)
2 EER = Estimated Energy Requirement. To meet energy needs, recommend using the EER calculations for age established by the Institute of Medicine (Dietary Reference Intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein and amino acids, 2005)
3 Include Physical Activity Level (PAL) in EER calculations for ages 4 to adult.
- PAL 4-18 years: Males - Sedentary = 1.0, Low Active = 1.12, Active = 1.27, Very Active = 1.45; Females - Sedentary =1.0, Low active = 1.18, Active = 1.35, Very active=1.6
- PAL ≥ 19 years: Males - Sedentary = 1.0, Low Active = 1.11, Active = 1.25, Very Active = 1.48; Females - Sedentary = 1.0, Low active = 1.12, Active = 1.27, Very active =1.45
4 EER for pregnancy
- Trimester 1 = EER for age
- Trimester 2 = EER pre-pregnancy + 340 kcal
- Trimester 3 = EER pre-pregnancy + 452 kcal
5 Protein requirements based on AI for infants 0-6 months of age and RDA for other age groups established by the Institute of Medicine (Dietary Reference Intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein and amino acids, 2005)
Type of Oil | Amount | Weight | Energy | Fat | LA1 | ALA2 |
Canola Oil | 1 | 0.9 | 8 | 0.9 | 170 | 80 |
Corn Oil | 1 | 0.9 | 8 | 0.9 | 480 | 10 |
Flaxseed Oil | 1 | 0.9 | 8 | 0.9 | 130 | 480 |
Peanut Oil | 1 | 0.93 | 8 | 0.93 | 300 | 0 |
Safflower Oil | 1 | 0.9 | 8 | 0.9 | 670 | 0 |
Sesame Oil | 1 | 0.9 | 8 | 0.9 | 290 | 0 |
Soybean Oil | 1 | 0.9 | 8 | 0.9 | 450 | 60 |
Walnut Oil | 1 | 0.9 | 8 | 0.9 | 480 | 90 |
1 Linoleic Acid
2 Alpha Linolenic Acid
Source: MetabolcPro © (https://metabolicpro.org), Accessed 10/10/18
| Domains1 | Infants | Children | Adults | Pregnancy and Postpartum Period | ||
| Measures | (0-1 year) | (1-7 years) | (8-18 years) | Adults | Pregnancy | Postpartum Lactation |
| Clinical Assessment | ||||||
| Nutrition Visit (dietary intake2 and nutrient analysis, nutrition-related physical findings, feeding skills, activity level, nutrition counseling) | Weekly to monthly | Monthly to every 6 months | Every 6 to12 months | Every 6 to12 months | Monthly to per trimester | At 6 weeks postpartum, then every 6 months while breastfeeding |
| Anthropometrics3 (weight, length or height, weight for length or BMI) | At every clinic visit3 Include head circumference | At every clinic visit3 Include head circumference through 36 months | At every clinic visit | At every clinic visit | At every clinic visit | At every clinic visit |
| Psychological | Routine neurodevelopmental assessments | |||||
| Biochemical (Routine) | ||||||
| Creatine Kinase | Every 3 months | Every 3 to 6 months | Every 6 months | At every clinic visit | At every clinic visit | At every clinic visit |
| Plasma carnitine (free, total, esterified) | Every 3 months | Every 6 months to yearly | Every 6 months to yearly | At every clinic visit | At every clinic visit | At every clinic visit |
| Biochemical (Conditional)4 | ||||||
| CMP (including LFT and blood glucose) Complete blood count, iron indices | As indicated | As indicated | As indicated | As indicated | As indicated | As indicated |
| Plasma acylcarnitines | Every 3 months | Every 3 to 6 months | Every 6 to12 mo | At every clinic visit | Weekly to monthly | At every clinic visit |
| For individuals on a long chain fat restriction: | ||||||
| Essential Fatty Acids (RBC or plasma) | Every 6 months | Every 6 months | Every 6 months | Every 6 months | Every 6 months | Every 6 months |
| 25-hydroxy- vitamin D, Vitamin E, Vitamin A | As indicated | As indicated | As indicated | As indicated | As indicated | As indicated |
| For individuals with a cardiac phenotype: | ||||||
| Troponin, B-natriuretic protein | As indicated | As indicated | As indicated | As indicated | As indicated | As indicated |
| Radiological | ||||||
| Dual-energy X-ray absorptiometry (DXA)5 | NA | NA | As indicated | Every 5 years | NA | NA |
| Echocardiogram | Initial evaluation, then as indicated by cardiologist | As indicated by cardiologist | As indicated by cardiologist | As indicated by cardiologist | As indicated by cardiologist | As indicated by cardiologist |
1Recommendations are for well individuals with a severe form of VLCAD and were derived from literature review and Delphi Survey. Frequency of clinical and laboratory assessments at a metabolic clinic (involving the medical geneticist, metabolic dietitian, social worker, nurse specialist, psychologist, genetic counselor, etc.) may not be possible because of travel distance, cost, loss of work days, etc. Coordination with primary care and community-based providers, use of telemedicine, and frequent communication by telephone and mail may be employed. More frequent monitoring may be necessary if individual is not in good metabolic control.
2A mechanism for assessing dietary intake, whenever blood is monitored, should be in place. MetabolicPro (http://www.metabolicpro.org) is a computer program available for dietary analysis of amino acid-restricted diets.
3The Centers for Disease Control and Prevention (CDC) recommends using the 2006 World Health Organization (WHO) Child Growth Standards to evaluate growth of infants ages birth to 24 months. The CDC recommends using the 2000 CDC Growth Charts (CDC) to evaluate the growth of children ages 2-20 years. Techniques for measurement are described on the CDC website (http://www.cdc.gov/growthcharts/cdc_charts.htm).
4Monitoring is indicated when nutrition assessment indicates a low fat intake, poor adherence to diet, and/or inadequate medical food consumption, or when there are clinical signs/symptoms of nutritional inadequacy including poor growth, or serious intercurrent illness. If laboratory values are abnormal, reassessment should be scheduled within one month of intervention.
5DXA is indicated in individuals who have frequent fractures, and/or low serum 25-hydroxyvitamin D concentrations, or other indices indicating possible bone abnormalities.
NOTE: This table is a summary of published literature used to inform the nutrition recommendation.
Reference | Age/Phenotype | History of Symptomology | Remains Asymptomatic | ||
LCF 1 (% Energy) | MCT 2 (% Energy) | LCF 1 (% Energy) | MCT 2 (% Energy) | ||
1-5 years | 10 | 20-25 | 15-20 | 15-20 | |
> 5 years | 10 | 20-25 | "Heart Healthy"3 | Use only in times of need (i.e. exercise) | |
Severe phenotype >12 months | 10 | >20 | 10 4 | >20 4 | |
Mild phenotype >12 months | "Heart Healthy"3 | Supplement with MCT5 | "Heart Healthy"3 | Supplement with MCT5 | |
1 LCF = long chain fat from diet sources, expressed as percent of total energy.
2 MCT = medium chain triglyceride supplementation, expressed as percent of total energy.
3 A "Heart Healthy" diet provides approximately 30% of total energy from fat sources.
4 In F.9, consensus was not reached for this recommendation, but majority response is provided.
5 Add a source of MCT to the diet; amount not specified.
NOTE: This table is a summary of published literature used to inform the nutrition recommendations.
Reference | Age/Phenotype | Symptomatic | Asymptomatic |
0-6 months | 100% MCT formula1 | Breast milk2 +/- MCT formula | |
6-12 months | MCT formula Low fat diet with 10% of energy from LCF | Breast milk +/- MCT formula Low fat diet with 15% of energy from LCF | |
0-4 months | 100% MCT formula | 50% Breast milk 50% MCT formula | |
> 4 months | Total fat (LCF + MCT): 25-30% of energy3 20% of energy as MCT | Total fat (LCF + MCT): 30-40% of energy 10-15 % of energy as MCT | |
Breastfeeding | |||
Severe phenotype < 12 months | Change to MCT formula | No consensus. Majority: Continue breast feeding as long as infant remains asymptomatic with normal cardiac function | |
Mild phenotype < 12 months | Continue breast feeding Supplement with source of MCT | Continue breast feeding | |
Bottle Feeding | |||
Severe phenotype < 12 months | Change to MCT formula | Change to MCT formula | |
Mild phenotype < 12 months | Change to MCT formula | No consensus to change to MCT formula | |
1 MCT formula = a medical food restricted in long chain fat (LCF) and supplemented with medium chain triglycerides (MCT). (RECOMMENDATION TABLE #5, Medical Foods for the Nutrition Management of VLCAD)
2 Breast milk from the breast or expressed breast milk
3 Total fat = long chain fat from diet + MCT supplement
NOTE: This table is a summary of published literature used to inform the nutrition recommendations.
| Presenting symptom(s) | Number of cases | Percent of patients prescribed MCT | References |
| Cardiomyopathy only | 14 | 100 | F.3712, F.4118, F.660, F.657, F.635, F.626, F.3797, F.4098, F.633, F.4124, F.18, F.15, F.4422, F.656 |
| Rhabdomyolysis only | 25 | 44 | F.3851, F.7, F.6, F.4052, F.621, F.4049, F.4380, F.4, F.4417, F.622, F.4164, F.4117, F.4051. F.3858, F.3851, F.630, F.17, F.4397, F.3719, F.636, F.658 |
| Cardiomyopathy and Rhabdomyolysis | 10 | 90 | F.14, F.3884, F.3896, F.654, F.4382 |
| Cardiomyopathy, rhabdomyolysis and hypoglycemia | 20 | 80 | F.4140, F.4415, F.4, F.3896, F.3735, F.3717 |
NOTE: This table is a summary of published literature used to inform the nutrition recommendations.
Age1 | Linoleic Acid (LA) (% of total energy) | α-Linolenic Acid (ALA) (% of total energy) |
0 to 4 months | 4.0 | 0.5 |
4 to 12 months | 3.5 | 0.5 |
1 to 4 years | 3.0 | 0.5 |
Over 4 years | 2.5 | 0.5 |
1 Source: (F.3)