|
Median age (years, (range))
|
3.6 (0.6-15.9)
|
6.13 (1.0-9.4)
|
14.4 (12.9-15.9)
|
4.1 (0.5-16.7)
|
|
N-Acetylaspartate
|
5.03 (0.53)
|
3.89 (0.20)†#
|
5.35 (0.28)
|
5.21 (0.51)
|
|
Creatine
|
4.57 (0.47)
|
3.71 (0.58)† $
|
4.42 (0.14)
|
4.55 (0.59)
|
|
Glutamine
|
1.29 (0.30) *
|
1.00 (1.00)*
|
1.27 (0.25)
|
1.98 (0.79)
|
|
Glutamate
|
4.28 (0.25)
|
3.07 (0.60)†#
|
4.30 (0.39)
|
4.45 (0.78)
|
|
Glutamine+Glutamate
|
5.57 (0.41) *
|
4.07 (1.19)† $
|
5.57 (0.28)
|
6.43 (1.39)
|
|
myo-Inositol
|
2.58 (0.26)
|
2.79 (0.90)
|
2.39 (0.0)
|
2.49 (0.43)
|
|
Lactate
|
0.21 (0.25)
|
0.93 (0.34)†#
|
0.00 (0.32)
|
0.11 (0.20)
|
|
Choline
|
1.10 (0.18)
|
1.16 (0.29)
|
0.90 (0.63)
|
1.11 (0.18)
|
|
White matter metabolites (mM, mean (standard deviation))
|
| |
Propionic Acidaemia
|
Normal MRI comparator group
|
| |
Metabolic stability (5 studies from 5 patients)
|
Acute encephalopathy (4 studies from 3 patients)
|
Post-liver transplant (2 studies from 2 patients)
|
(53 studies)
|
|
Median age (years, (range))
|
3.1 (0.6-15.9)
|
7.74 (3.0 -9.4)
|
14.4 (12.9-15.9)
|
4.7 (0.5-16.7)
|
|
N-Acetylaspartate
|
5.28 (0.80)
|
4.50 (0.47)
|
6.11 (0.39)
|
5.11 (0.73)
|
|
Creatine
|
3.55 (0.19) *
|
3.33 (0.53)
|
3.70 (0.00)
|
3.18 (0.47)
|
|
Glutamine
|
1.43 (0.54)
|
1.34 (1.30)
|
1.62 (0.49)
|
1.32 (0.62)
|
|
Glutamate
|
3.83 (0.45)
|
2.65 (0.44)*$
|
4.27 (0.31)
|
3.51 (0.78)
|
|
Glutamine+Glutamate
|
5.26 (0.89)
|
3.98 (1.54)
|
5.89 (0.80)
|
4.84 (1.18)
|
|
myo-Inositol
|
2.73 (0.31)
|
2.57 (0.39)
|
2.79 (0.02)
|
2.57 (0.39)
|
|
Lactate
|
0.40 (0.30)
|
0.81 (0.74)*
|
0.32 (0.45)
|
0.21 (0.28)
|
|
Choline
|
0.93 (0.15)*
|
1.12 (0.37)
|
0.83 (0.09)
|
1.08 (0.15)
|
