Skip to main content

Table 2 Nutrition management

From: The efficacy of Carbamylglutamate impacts the nutritional management of patients with N-Acetylglutamate synthase deficiency

Case

Diet during initial hyper-ammonemic crisis

Pre-carbamylglutamate diet

1 Age at Dietary Record

Calories (kcal/kg/day)

Protein (g/kg/day)

Arginine (mg/kg/day)

Citrulline (mg/kg/day)

3 days

68

1

250

0

39 days

117

1.6

0

200

2 Age at Dietary Record

Calories (kcal/kg/day)

Protein (g/kg/day)

Arginine (mg/kg/day)

Citrulline (mg/kg/day)

6 days

120

0

0

0

24 days

130

1.0

250

0

3 Age at Dietary Record

Calories (kcal/kg/day)

Protein (g/kg/day)

Arginine (mg/kg/day)

Citrulline (mg/kg/day)

38 years

46

1.4

0

0

39 years

Not available

1.0

0

50

4 Age at Dietary Record

Calories (kcal/kg/day)

Protein (g/kg/day)

Arginine (mg/kg/day)

Citrulline (mg/kg/day)

25 months

122

1.2

0

170

28 months

64

1.05–1.2

0

160

5 Age at Dietary Record

Calories (kcal/kg/day)

Protein (g/kg/day)

Arginine (mg/kg/day)

Citrulline (mg/kg/day)

14 years

35

0

0

150

18 years

30

0.5

0

150

6 Age at Dietary Record

Calories (kcal/kg/day)

Protein (g/kg/day)

Arginine (mg/kg/day)

Citrulline (mg/kg/day)

10 years

17

0

114

0

18 years

18.8

0.47

0

130

7 Age at Dietary Record

Calories (kcal/kg/day)

Protein (g/kg/day)

Arginine (mg/kg/day)

Citrulline (mg/kg/day)

2 years

28

0.36

0

210

22 years

28

0.45

0

200