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Table 2 Protein and energy intake, body mass index, and plasma metabolites before and after initiating continuous treatment with N-carbamylglutamate

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

Parameter

Before NCG (mean ± SD)

During NCG (mean ± SD)

p Value (two-tailed paired t test)

Natural protein intake (g/kg/day)

0.7 ± 0.12

0.85 ± 0.18

n.s.

Energy intake (kcal/kg)

67.3 ± 13.2

56.2 ± 19.1

n.s.

BMI (kg/m2)

14.3 (< 10th percentile)

18.4 (> 25th percentile)

n.s.

Metabolites (μmol/L, number of measurements)

 Ammoniaa

140.3 ± 47.2, 18

75.7 ± 37, 11

0.003

 Glutamineb

297.3 ± 64.8, 8

296 ± 160, 12

0.4

 Alaninec

430.3 ± 123.9, 5

596.8 ± 318.9, 10

0.09

 Glycined

561.8 ± 496.2, 7

1349 ± 496.9, 12

0.01

  1. Abbreviations: BMI Body mass index, NCG N-carbamylglutamate, n.s. Not significant, SD standard deviation
  2. aNormal range 50–80 μmol/L
  3. bNormal range 420–730 μmol/L
  4. cNormal range 142–421 μmol/L
  5. dNormal range 60–404 μmol/L