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MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.55
Current version: v.2.0
Updated: June 2021
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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 Dietary PRO, BCAA and Energy Intake 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 Dietary PRO, BCAA and Energy Intake

AGE/

STAGE

NUTRIENT

LEU 1,2

mg/kg

Approximate

Intact PRO5

g/kg

ILE2,3,4

mg/kg

VAL2,3,4

mg/kg

Total PRO6 g/kg

ENERGY7 kcal/kg

INFANTS TO < 4 yr (classical to mild MSUD)

0 - <3 mo

60-100

1.0-1.6

36-100

40-95

2.5-3.0

118-130

>3 - <6 mo

50-85

0.8-1.4

30-80

35-90

2.0-3.0

102-111

>6 - <12 mo

35-70

0.6-1.2

25-70

30-80

2.0-2.5

100-107

1 - <4 yr

25-55

0.4-0.9

20-60

25-70

1.5-2.1

105-114

AGE/

STAGE

AFTER EARLY CHILDHOOD (classical MSUD)

LEU2

mg per day

Intact PRO5

g per day

ILE3,4

mg per day

VAL3,4

mg per day

Total PRO6 g

ENERGY

kcal

>4 yr

275-500

5.0 - 8.0

250-450

325-500

120% DRI8

DRI8

AGE/

STAGE

PREGNANCY9,10 and POSTPARTUM (classical MSUD)

LEU3

mg per day

Intact PRO5

g per day

ILE3,4

mg per day

VAL3,4

mg per day

Total PRO6

g/day

ENERGY

kcal/day

1st trimester10

300 -500

5.0 - 8.0

250-450

325-500

120% DRI

plus 0.5g/d

DRI plus

85 kcal/d

2nd trimester

600-1000

10 - 16

400-800

~600-1000

~120 % DRI

plus 7.7g/d

DRI plus

285 kcal/d

3rd trimester

800-2000

13 - 33

650-1200

800-1800

120% DRI

plus 25g/d

DRI plus

375 kcal /d

Postpartum

If not breast feeding, return to pre-pregnancy intake 11

Lactation

Intake while breastfeeding should be approximately the same as in the 3rd trimester

Sources: G.43; G.50; G.39; F.426; F.3481; F.102; F.3482; F.3483, Nominal Group summary and Delphi Surveys

1 For individuals with classical MSUD, especially in infancy, use of LEU content of foods/infant formulas is more accurate than relying of the rounded PRO content.

2 Individuals with "classical MSUD" tolerate BCAA and intact PRO intake at the lower end of the range.

3 Actual intake should be guided and adjusted based on results from plasma amino acid and anthropometric monitoring.

4 Source(s) of VAL and ILE are from intact PRO and possible supplementation of (the individual) amino acids.

5 Estimated from a mixed diet to be approximately 60mg LEU per gram intact PRO

6 Combination of intact PRO (providing the increased requirements for BCAA) and PRO equivalents from amino acid-based medical food

7 Energy intake recommendations from birth to <4 years are derived from the Human Energy Requirements Report of a Joint FAO/WHO/UNU Expert Consultation 2001 for the general public - see: http://www.fao.org/3/a-y5686e.pdf with an additional 10% added for those individuals who obtain the majority of their protein calories from amino acids (G.50)

8 DRI (Daily Intake Requirements) are based on age, sex, activity level and BMI - see:

https://fnic.nal.usda.gov/dietary-guidance/dri-nutrient-reports/energy-carbohydrate-fiber-fat-fatty-acids-cholesterol-protein.

9 Women who are < 19 years of age and women having multiple births have higher nutrient requirements

10 Women may need to adjust their current intake to meet appropriate plasma levels of the BCAA if diet was not being strictly followed at the time of conception. BCAA, PRO and energy needs begin to increase toward the end of the first trimester

11 See MSUD guideline's text for recommendations for the labor/delivery and immediate post-partum periods

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