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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

source=   http://frontalcortex.com/?page=forums&topic=user_forums&qid=f12


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