Table 2.
Intake of amino acids in subjects with PKU consuming a low-Phe diet in combination with AA-MF and GMP-MF.
| Medical Foods (g/day) | Natural Foods (g/day) | Whole Diet (g/day) | |||||||
|---|---|---|---|---|---|---|---|---|---|
| AA-MF | GMP-MF | p | AA-MF | GMP-MF | p | AA-MF | GMP-MF | p | |
|
|
|||||||||
| Phenylalanine | 0.00 ± 0.00 | 0.09 ± 0.01 | <0.001 | 1.06 ± 0.20 | 1.19 ± 0.22 | 0.443 | 1.06 ± 0.20 | 1.29 ± 0.22 | 0.191 |
| Tryptophan | 1.28 ± 0.10 | 0.81 ± 0.04 | <0.001 | 0.39 ± 0.18 | 0.41 ± 0.14 | 0.811 | 1.67 ± 0.18 | 1.22 ± 0.14 | <0.001 |
| Tyrosine | 6.06 ± 0.39 | 4.18 ± 0.36 | <0.001 | 1.14 ± 0.55 | 1.22 ± 0.43 | 0.725 | 7.20 ± 0.55 | 5.39 ± 0.51 | <0.001 |
| LNAA | 28.6 ± 1.84 | 31.1 ± 1.87 | 0.244 | 7.33 ± 2.14 | 8.35 ± 1.90 | 0.445 | 35.9 ± 2.19 | 39.4 ± 2.05 | 0.126 |
| Isoleucine | 3.71 ± 0.26 | 3.89 ± 0.22 | 0.543 | 0.97 ± 0.24 | 1.13 ± 0.23 | 0.366 | 4.68 ± 0.27 | 5.02 ± 0.22 | 0.281 |
| Leucine | 6.60 ± 0.45 | 10.8 ± 0.63 | <0.001 | 1.69 ± 0.37 | 2.04 ± 0.40 | 0.297 | 8.29 ± 0.41 | 12.8 ± 0.57 | <0.001 |
| Threonine | 3.24 ± 0.26 | 5.95 ± 0.36 | <0.001 | 0.86 ± 0.21 | 0.99 ± 0.19 | 0.387 | 4.09 ± 0.29 | 6.95 ± 0.32 | <0.001 |
Values are mean ± SEM based on 3-d food record, n=18. Large neural amino acids (LNAA) include Tyr, Trp, Leu, Ile, Val, His, Met and Thr. AA medical foods (AA-MF), GMP medical foods (GMP-MF). Natural foods are defined as all foods and beverages that are not medical foods.