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Urinary KGM (mmol/mol creatinine) and blood ammonia levels in patients with inborn errors of metabolism resulting in secondary hyperammonemia
| Patient ID | Defect | Age | KGM | Blood NH3 (μM) |
|---|---|---|---|---|
| PCCD1 | Propionic acidemia (severe) | 3 days | 2.99 | 206 |
| PCCD1 | Propionic acidemia | 42 days | 4.00 | >235 |
| PCCD2 | Propionic acidemia(mild) | 6 days | 1.48 | <40 |
| MMA1 | Methylmalonic acidemia | 1 year 4 months | 4.19 | 63 |
| MMA1 | Methylmalonic acidemia | 1 year 5 months | 2.75 | 53 |
| MMA2 | Methylmalonic acidemia | 1 year 1 month | 2.59 | <40 |
