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| IEM | Abnormal odor |
|---|---|
| Maple syrup urine disease (MSUD) | Maple syrup |
| Isovaleric acidemia or glutaric acidemia type II | Sweaty feet |
| Cystinuria and tyrosinemia type I | Sulfur |
| Tyrosinemia type I | Boiled cabbage |
| Trimethylaminuria or dimethylglycine dehydrogenase deficiency | Old fishy |
| Multiple carboxylase deficiency (MCD) | Cat’s urine |
| Phenylketonuria | Mousy |
IEM, inborn error of metabolism.
