Comparative standards are reference values from reputable and authoritative sources such as government agencies, independent scientific panels, standards organizations, and scientific and professional organizations. See TABLE #4, Recommended Daily Nutrient Intakes of BCAA, PRO, ENERGY and Fluids for Infants, Children and Adults with MSUD (when well) for more information.
Comparison of nutrient intake to established standards of the DRI. See: http://fnic.nal.usda.gov/dietary-guidance/dietary-reference-intakes/dri-tables
TABLE 3 - Nutrient Recommended Intake and Sources in the Dietary Treatment of Well Individuals with MSUD
Nutrient | Recommendation | Source |
LEU | Sufficient intake to allow adequate protein synthesis for growth, repair and health maintenance and to achieve LEU levels in recommended treatment range. LEU allowance is also dependent on residual BCKD activity, age, weight, sex, life stage and health of the individual with MSUD. In the newborn, the recommended intake is: 40-100 mg LEU/kg/day |
In infants: breast milk or infant formula with known LEU content In children and adults: foods such as fruits/vegetables, some grains/cereals that are typically low in protein and for which there is known LEU content |
PRO | DRI 1 Plus additional 20-40% if an amino acid-based medical food is used |
|
VAL, ILE | VAL and ILE are essential amino acids and may need to be supplemented when BCAA are restricted to achieve appropriate LEU blood levels. To promote anabolism of LEU, when LEU blood levels are high, additional supplementation of VAL and ILE is often required |
|
KCAL | DRI 1 |
|
Other nutrients, minerals and vitamins 5 | DRI 1 |
|
1 For age, size, sex, and life stage. Requirements change with catabolic illness/conditions
2 1% solutions are convenient for adding to the medical food when supplementation is necessary
3 Free foods contain little or no detectable PRO/BCAA and consist mostly of sugars, pure starches and/or fats
4 Modified low-protein foods include pastas and baked goods where higher protein grains/flours are replaced by protein-free starches
5 Included are essential fatty acids and DHA, Vit D, Vit A, Ca, Fe, Zn, Se
6 Most BCAA-free medical foods are supplemented sufficiently with the nutrients and micronutrients that may be deficient in a diet low in BCAA. Compliance with taking the full medical food prescription is important in meeting these nutrient requirements. In addition, there are some medical foods that have been modified to improve taste, decrease KCAL or volume in order to increase compliance that may have insufficient supplementation of some micronutrients, vitamins and minerals
TABLE 4 - Recommended Daily Nutrient Intakes of BCAA, PRO, ENERGY and Fluids for Infants, Children and Adults with MSUD (when well)
AGE | NUTRIENT | |||||
LEU mg/kg | ILE mg/kg | VAL mg/kg | PROTEIN g/kg | ENERGY kcal/kg | FLUID ml/kg | |
0 to 6 mo | 40-100 | 30-90 | 40-95 | 2.5-3.5 | 95-145 | 125-160 |
6 to 12 mo | 40-75 | 30-70 | 30-80 | 2.5-3.0 | 80-135 | 125-145 |
1-3 yr | 40-70 | 20-70 | 30-70 | 1.5-2.5 | 80-130 | 115-135 |
4-8 yr | 35-65 | 20-30 | 30-50 | 1.3-2.0 | 50-120 | 90-115 |
9-13 yr | 30-60 | 20-30 | 25-40 | 1.2-1.8 | 40-90 | 70-90 |
14-18 yr | 15-50 | 10-30 | 15-30 | 1.2-1.8 | 35-70 | 40-60 |
19 yr + | 15-50 | 10-30 | 15-30 | 1.1-1.7 | 35-45 | 40-50 |
TABLE 5 - Protein Recommendations during Pregnancy for MSUD
Ref | Trimester | Total protein (g/kg body weight) | Intact protein (g/kg body weight) | BCAA-free protein (g/kg body weight) |
Pre-pregnancy | 1.0 – 1.2 g | 0.6 – 0.8 g | 0.4 g | |
First trimester | 1.2 g | 0.6 g | 0.6 g | |
Second trimester | ~ | 0.8 g1 | ~ | |
Postpartum | ~ | 1.0 g | ~ | |
First trimester | 1.1 g | 0.1 g | 0.9 g | |
Second trimester | 1.5 g | 0.4 g | 1.1 g | |
Third trimester | 1.1 g | 0.4 g | 0.8 g | |
L.8 2 | Lactation | 1.1 g | 0.4 g | 0.8 g |
1 Increased to normalize low plasma BCAA levels: ~ not specified
2 Nutrient requirements during lactation are similar to those of the third trimester