Useful Info

All life requires protein since it is the chief tissue builder and part of every cell in the body. Among other functions, proteins help to: make hemoglobin in the blood that carries oxygen to the cells; form anti-bodies that fight infection; supply nitrogen for DNA and RNA genetic material; and supply energy.

Proteins are necessary for nutrition because they contain amino acids. Among the 20 or more amino acids, the human body is unable to synthesize 8, therefore, these amino acids are called essential amino acids. A food containing protein may be of poor biological value if it is deficient in one or more of the 8 essential amino acids: lysine, tryptophan, methionine, leucine, isoleucine, phenylalanine, valine, and threonine. Proteins of animal origin have the highest biological value because they contain a greater amount of the essential amino acids. Foods with the best quality protein are listed in diminishing quality order: whole eggs, milk, soybeans, meats, vegetables, and grains.


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


Gene&Gene Mutation

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Body regulates thousands of metabolic reactions simultaneously

During Gestation

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Over the last 3 decades many new and effective therapies.....

Inborn Errors of Metabolism in Infancy: A Guide to Diagnosis

Metabolism Summary

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