Diet prescription - MSUD

Establishing a prescription is done in a stepwise manner:

Step 1	Fill the BCAA requirements through the use of measured quantities of intact protein from breast milk or regular infant formula in infants and by natural food intake beyond infancy
Step 2	Fill the protein requirement by adding the BCAA free–L-amino acids supplement that provides a LEU, VAL ILE free mixture of amino acids, and other nutrients
Step 3	Fill energy requirements by adding non-protein calorie sources as needed. Energy needs should be based on ideal body weight whenever possible and are the same as for non-MSUD patients
Step 4	Adjust fluid volume to meet requirements
Step 5	Calculate vitamin, mineral and trace-element content of the final mixture, adjust or supplement as needed

Dietary recommendations:

Protein recommendations

For healthy individuals, protein recommendations are based on the rates of growth, protein deposition for children of different age groups, the amino acid composition of whole body protein, as well as the efficiency of dietary protein utilization¹ and more recently by a stable isotopic technique².

The optimal protein intake is not agreed, where some will allow an inefficiency factor for L-amino acid sources of 1.2³.

Amino acids do not require digestion and are directly available for absorption by the small intestine, which leads to rapid absorption and cause a transient imbalance of amino acids in the systemic circulation as opposed to whole protein intake^{4, 5}.

ESPKU preliminary recommendations for Protein in PKU, can also be applicable for MSUD:

Total protein intake should supply age-related safe levels of protein intake, based on WHO/FAO/UNU2007, with an additional 20% of L-aminoacids to compensate for the ‘digestible indispensable amino acid score and further 20% to optimize impact of L-AA on blood Phe control.

Clinical practice differs throughout the world; no consensus exists on protein substitute dosage.

Protein recommendations for MSUD, based on preliminary report of the ESPKU recommendations for protein in PKU:

Age	gProtein/kg bodyweight
0-2 months	2,5
2-12 months	2,0
1-4 years	1,5
4-10 years	1,2
10-14 years	1,1
>14 years	1,0

Energy requirements infants		Energy requirements children
Age/months	Kcal/kg/d	Age years	Kcal/kg/d – M	Kcal/kg/d – F
1	110	1-2	82	80
2	102	2-3	84	81
3	95	3-4	80	77
4	83	4-5	77	74
5	82	5-6	75	72
6	81	6-7	73	69
7	79	7-8	71	67
8	79	8-9	69	64
9	79	9-10	67	61
10	80	10-11	65	58
11	80	11-12	62	55
12	80	12-13	60	52
		13-14	58	49
		14-15	56	47
		15-16	53	45
		16-17	52	44
		17-18	50	44
		<18

Human energy requirements
Report of a joint FAO/WHO/UNU Expert consultation. October 2001

Fluid requirements, based on requirements of healthy population
Age	Weight (kg)	Recommendation (ml)
0-3 months		150 x weight (kg)
4-6 months		130 x weight (kg)
7-9 months		120 x weight (kg)
10-12 months		110 x weight (kg)
>1 year	1-10	100 x weight (kg)
	11-20	1000 + 50 x (weight in kg – 10)
	> 20	1000 + 20 x (weight in kg – 20)

Human fluid requirements
Report of a Joint FAO/WHO/UNU expert consultation 2001

% of leucine, valine and Isoleucine found protein of milk, cereals, vegetables and fruits⁷
Protein source	% Leucine found	% Valine found	% Isoleucine found
Milk, cereals	10-11%	6-7 %	4-6%
Vegetables	3-6 %	4-5 %	3-5%
Fruits	5 %	3-4 %	3-4%

References

Pillai RR, Kurpad AV Amino acid requirements in children and the elderly population. Br J Nutr, 2012, 108(Suppl 2) S44−9.
Lango R, Levesque C, Ball RO et al. Available versus digestible amino acids − new stable isotope methods. Br J Nutr, 2012, 108(Suppl 20) S306−14.
Gropper SS, Acosta PB Effect of simultaneous ingestion of L-amino acids and whole protein on plasma amino acid and urea nitrogen concentrations in humans. J Parenter Enteral Nutr, 1991;15:48−53
Gropper SS, Smith JL Advanced Nutrition and Human Metabolism. California, USA: Wadsworth Cengage Learning, 2013;183−244.
van Rijn G, Carbasius Weber E. Dieet bij metabole ziekten 1st Ed. WKZ/UMCU Utrecht. 2011:24-38.
Human energy requirements, report of a joint FAO/WHO/UNU Expert consultation. October 2001
Dixon MD, MacDonald A, White F, Stafford J. In: Clinical Paediatric Dietetics. 4^th ed: West Sussex: John Wiley & Sons Limited, 2014:422.