Recommended Nutrient Intakes

A list of recommended intakes for initiating and managing nutrition therapy in selected patients with inborn errors.

RECOMMENDED NUTRIENT INTAKES

A list of recommended intakes for initiating and managing nutrition therapy in selected patients with inborn errors.

Nutrients

Age (years)

 

0.0 < 0.5

0.5 < 1.0

1 < 4

4 < 7

7 < 11

11 < 19

> 19 years

INHERITED DISORDERS OF AMINO ACID METABOLISM

Phenylketonuria/ Hyperphenylalaninemia

PHE, mg

70-25/kg

35-15/kg

200-450/day

225-450/day

250-500/day

300-1000/day

300-750/day

TYR, mg

300-250/kg

250-225/kg

1720-3000/day

2250-3500/day

2500-4000/day

2100-5500/day

3500-6500/day

Protein, g

3.5-3.0 kg

3.0-2.5/kg

≥30/day

≥35/day

≥40/day

50-65/day

50-65/day

Energy, kcal

                  100%-125% OF NAS/FNB RDA for age 

Branched-Chain Amino Acids

Maple syrup disease

             

ILE, mg

75-35/kg

65-30/kg

165-325/day

225-420/day

250-470/day

330-570/day

300-700/day  *

LEU, mg

100-50/kg

75-35/kg

275-535/day

360-695/day

410-785/day

540-945/day

400-1100/day

VAL, mg

80-40/kg

70-30/kg

200-375/day

250-500/day

285-550/day

375-675/day

420-800/day  *

Protein, g

3.5-3.0/kg

3.0-2.5/kg

≥30/day

≥35/day

≥40/day

50-65/day

50-65/day

Energy, kcal

                  100%-125% OF NAS/FNB RDA for age 

Organic Acidemias (Selected)

Isovaleric acidemia

LEU, mg

130-70/kg

120-50/kg

500-900/day

600-900/day

700-900/day

700-1500/day

820-1700/day

L-carnitine mg

50-100/kg

         

50-100/kg

GLY, mg

150-300/kg

         

150-300/kg

Protein, g

3.0-2.5/kg

3.0-2.5/kg

≥30/day

≥35/day

≥40/day

50-65/day

50-65/day

Energy, kcal

                  100%-125% OF NAS/FNB RDA for age 

Propionic acidemia and methylmalonic acidemia

ILE, mg

110-60/kg

90-40/kg

485-735/day

630-960/day

715-1090/day

956-1470/day

925-1470/day

MET, mg

50-20/kg

40-15/kg

275-390/day

360-510/day

410-580/day

550-780/day

530-900/day

THR,mg

125-50/kg

75-20/kg

415-600/day

540-780/day

610-885/day

830-1200/day

800-1350/day

VAL, mg

105-60/kg

80-40/kg

550-830/day

720-1080/day

815-1225/day

1105-1655/day

1000-1850/day

D-Biotin, mg

                  5-10/day for propionic academia   

Hydroxycobalamin, mg (oral)

                  1-2/day for cobalamin-responsive methylmalonic academia 

L-Carnitine, mg

300-100/kg

         

100-300/kg

Protein,† g

3.0-2.5/kg

3.0-2.5/kg

≥30/day

≥ 35/day

~40/day

~50/day

~50/day

Energy, kcal

                  100%-125% OF NAS/FNB RDA for age 

UREA CYCLE DISORDERS

Citrullinemia; Argininosuccinic aciduria

ARG, mg

700-350/kg

700-350/kg

500-250/kg

500-250/kg

500-250/kg

400-200/kg

400-200/kg

Protein,‡ g

2.2-1.7/kg

1.8-1.5/kg

8-12/day

12-15/day

14-17/day

20-24/day

20-28/day

Energy, kcal

                  115-140% OF NAS/FNB RDA for age     

Carbamylphosphate synthetase deficiency; Ornithine transcarbamylase deficiency

CIT, mg

700-350/kg

700-350/kg

500-250/kg

500-250/kg

500-250/kg

400-200/kg

400-200/kg

Protein,‡ g

2.2-1.7/kg

1.8-1.5/kg

8-12/day

12-15/day

14-17/day

20-24/day

20-28/day

Energy

                  115-140% OF NAS/FNB RDA for age     

Argininemia

Protein,‡ g

2.2-1.7/kg

1.8-1.5/kg

8-12/day

12-15/day

14-17/day

20-32/day

20-32/day

Energy, kcal

                  115%-140% of NAS/FNB RDA for age