Unusual Smell In Urine?
Many inborn errors of metabolism are associated with unusual smells in the urine. These often go unnoticed by the
physician, but are detected by a nurse or a parent. These odors may have significant diagnostic value.
The description of the odor will vary depending on the cultural background of the person describing the odor.
There are some urine odors which are described as being associated with specific inborn errors:
| Disorders | Smells |
| Maple syrup urine disease (MSUD) | curry, maple syrup, burnt sugar, or fenugreek. |
| Phenylketonuria (PKU) | musty or mousy smell |
| Cystinuria | sulfur smell |
| Isovaleric acidemia, Multiple acyl-Coa dehydrogenase deficiency, and 3-hydroxy-3-methylglutaric aciduria | sweaty feet smell |
| Methionine malabsorption | cabbage smell |
| Tyrosinemia | cabbage or rancid butter. |
| Trimethylaminuria | fish market smell |
| Methylmalonic academia | acidic odor |
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