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