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Table 1 Number of admissions, mean ammonia levels, and protein intake, according to patient age

From: Long-term continuous N-carbamylglutamate treatment in frequently decompensated propionic acidemia: a case report

Age (years)

Number of admissions

Ammonia levelsa, mean (±SD) (μmol/L)

Protein (natural proteins) intake (g/kg/day)

0–1

9

121 ± 14.7

1.5 (1)

1–2

10

116 ± 35.4

1.6 (0.9)

2–3

8

131 ± 12.4

1.4 (0.8)

3–4

10

89 ± 17.9

1.1 (0.7)

4–5

7

128 ± 27.3

1 (0.6)

5–6

8

96 ± 17.7

1 (0.5)

6–7

9

112 ± 30.5

1.1 (0.6)

7–8

9

199 ± 70.6

0.95 (0.6)

8–9

8

114 ± 31.8

0.9 (0.5)

9b–10

2

69 ± 21.5

1 (0.7)

10–11

0

74 ± 30.4

1.1 (0.8)

11–15

0

61.7 ± 48

1.2 (1)

  1. Abbreviations: NCG N-carbamylglutamate, SD standard deviation
  2. aNormal range 50–80 μmol/L
  3. bContinuous treatment with oral NCG 100 mg/kg/day (50 mg/kg/day after 6 months) initiated