Table 1.
Age | Centres using PFAA |
Centres not using PFAA |
||||||||
---|---|---|---|---|---|---|---|---|---|---|
0-6 m n = 4 |
7-12 m n = 6 |
1-10y n = 64 |
11-16y n = 37 |
> 16y n = 33 |
0-6 m n = 0 |
7-12 m n = 3 |
1-10y n = 21 |
11-16y n = 11 |
> 16y n = 7 |
|
Total protein (g/kg) | ||||||||||
Median | 2.2 | 1.9 | 1.6 | 1.2 | 1.0 | None reported | 1.0 | 1.0 | 1.0 | 1.0 |
Min | 1.6 | 1.1 | 1.0 | 0.5 | 0.7 | 1.0 | 0.8 | 0.5 | 0.6 | |
Max | 3.0 | 3.0 | 2.3 | 1.9 | 1.5 | 1.2 | 1.5 | 1.8 | 1.0 | |
Natural protein (g/kg) | ||||||||||
Median | 1.0 | 1.0 | 0.8 | 0.7 | 0.6 | None reported | 1.0 | 1.0 | 1.0 | 1.0 |
Min | 0.5 | 0.5 | 0.4 | 0.3 | 0.4 | 1.0 | 0.8 | 0.5 | 0.6 | |
Max | 1.3 | 1.2 | 1.4 | 1.2 | 0.8 | 1.2 | 1.5 | 1.8 | 1.0 | |
PFAA (g/kg) | ||||||||||
Median | 1.2 | 0.9 | 0.7 | 0.6 | 0.5 |
- |
||||
Min | 0.3 | 0.2 | 0.3 | 0.2 | 0.2 | |||||
Max | 2.5 | 2.5 | 1.8 | 0.9 | 0.8 | |||||
Amount of total protein from PFAA used (%) | ||||||||||
Median | 55 | 44 | 43 | 43 | 50 |
- |
||||
Min | 19 | 14 | 19 | 20 | 25 | |||||
Max | 83 | 83 | 78 | 70 | 67 |
PFAA: propionic acidaemia precursor-free amino acid supplements.
n: number of patients.