Useful Info
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:



Maple syrup urine disease (MSUD)

curry,   maple syrup ,  burnt sugar , or  fenugreek.  

Phenylketonuria (PKU)


  musty  or  mousy  smell


 sulfur  smell

Isovaleric acidemia, Multiple acyl-Coa dehydrogenase deficiency, and 3-hydroxy-3-methylglutaric aciduria


 sweaty feet  smell

Methionine malabsorption


 cabbage  smell



 cabbage  or  rancid butter.


 fish market  smell

Methylmalonic academia


acidic odor

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