Table 1. Concentrations of urine organic acids (mmole/mole creatine) at different age.
| Patient age: | 2-Methyl-3-hydroxybutyric acid | Tiglylglycine |
| 8 y 4 m | 31 | 32 |
| 8 y 8 m | 18 | 9 |
| 10 y 3 m | 152 | 66 |
| 10 y 4 m | 80 | 40 |
| Normal children (age: 2 to 12-year-old) | <14 | <6 |
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