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Table 1 Composition of the ready-to-drink protein substitute (1 pouch of 130 ml) used for the dietary management of the ECHS1 case

From: Clinical improvements after treatment with a low-valine and low-fat diet in a pediatric patient with enoyl-CoA hydratase, short chain 1 (ECHS1) deficiency

Energy (kcal)

103

Minerals

 

Total amino acids (g)

18.2

Sodium (mg)

127

Protein equivalent (g)

15.0

Potassium (mg)

235

Carbohydrates (g)1

7.0

Chlore (mg)

182

Sugars (g)

5.9

Calcium (mg)

299

Lipids (g)

1.7

Phosphorus (mg)

267

Saturated fat (g)

0.33

Magnesium v

94

DHA (mg)

100

Iron (mg)

5.4

EPA (mg)

23

Copper (mg)

0.55

Amino acids (g)

 

Zinc (mg)

5.4

Alanine

1.68

Manganese (mg)

0.8

Arginine

1.73

Iodine (μg)

63

Aspartic acid

3.0

Molybdenum (μg)

36

Cystine

0.73

Selenium (μg)

22

Glutamine

0.00

Chromium (μg)

22

Glycine

0.79

Vitamins

 

Histidine

1.13

A (μg)

208

Isoleucine

0.05

D (μg)

7.5

Leucine

2.37

E (mg)

3.9

Lysine

1.85

K (μg)

25

Methionine

0.00

C (mg)

27

Phenylalanine

1.13

Thiamine (B1) (mg)

0.50

Proline

0.83

Riboflavin (B2) (mg)

0.57

Serine

1.25

Niacin (mg) (B5)

6.2

Threonine

0.00

Pyridoxamine (B6) (mg)

0.70

Tryptophan

0.47

Folic acid (B9) (μg)

100

Tyrosine

1.13

B12 (μg)

1.2

Valine

0.00

Biotin (B7)

47

Carnitine (mg)

17

Pantothenic acid (B3) (mg)

2.0

Taurine (mg)

33

Choline (mg)

150

  1. EPA, eicosapentaenoic acid; DHA, docosahexaenoic acid
  2. aSucrose, glucose syrup, and modified corn starch