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MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.50
Current version: v.1.58
Updated: August 2016
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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. 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

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

  • Intact protein (PRO)

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

  • Intact PRO (as above)
  • BCAA-free medical food

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

  • Intact PRO
  • Supplemental VAL, ILE2

KCAL

DRI 1

  • Intact PRO
  • BCAA-free medical food
  • Free foods 3
  • Modified low PRO food 4

Other nutrients, minerals and vitamins 5

DRI 1

  • Intact PRO
  • BCAA-free medical food
  • Supplemental nutrients, vitamins and minerals 6

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)

G.50

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)

F.102

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

~

F.78

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 2Lactation1.1 g0.4 g0.8 g

1 Increased to normalize low plasma BCAA levels: ~ not specified

2 Nutrient requirements during lactation are similar to those of the third trimester

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