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the scent of urine

Topic: Adult

Created on Friday, September 14 2007 by jdmiles

Last modified on Sunday, September 16 2007.

Which of the following is most accurate regarding the scent of urine?

 
        A) Isovaleric acidemia is associated with a "maple syrup" smell.
 
        B) Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "sweaty feet" smell.
 
        C) 3-hydroxy-3-methylglutaric aciduria is associated with a "maple syrup" smell.
 
        D) Phenylketonuria is associated with a "fish market" smell.
 
        E) Maple syrup urine disease is associated with a "cat urine" smell.
 

 


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This question was created on September 14, 2007 by jdmiles.
This question was last modified on September 16, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) isovaleric acidemia is associated with a "maple syrup" smell.

This answer is incorrect.


The statement, "isovaleric acidemia is associated with a "maple syrup" smell." is false.
isovaleric acidemia is associated with a "sweaty feet" smell.  (See References)

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B) multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "sweaty feet" smell.

This answer is correct.


The statement, "multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "sweaty feet" smell." is true.
multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "sweaty feet" smell.  (See References)

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C) 3-hydroxy-3-methylglutaric aciduria is associated with a "maple syrup" smell.

This answer is incorrect.


The statement, "3-hydroxy-3-methylglutaric aciduria is associated with a "maple syrup" smell." is false.
3-hydroxy-3-methylglutaric aciduria is associated with a "sweaty feet" smell.  (See References)

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D) phenylketonuria is associated with a "fish market" smell.

This answer is incorrect.


The statement, "phenylketonuria is associated with a "fish market" smell." is false.
phenylketonuria is associated with a "musty" or "mousy" smell.  (See References)

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E) maple syrup urine disease is associated with a "cat urine" smell.

This answer is incorrect.


The statement, "maple syrup urine disease is associated with a "cat urine" smell." is false.
maple syrup urine disease is associated with a "curry" smell, is associated with a "fenugreek" smell, is associated with a "maple syrup" smell, and is associated with a "burnt sugar" smell.  (See References)

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References:

1. Pastores, G.M. and Kolodny, E.H. (2004). Inborn errors of metabolism of the nervous system. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 1811-1832.
2. Burton, B.K. (1998). "Inborn errors of metabolism in infancy: a guide to diagnosis." Pediatrics, 102(6) E69. (PMID:9832597)
3. Monastiri, K., Limame, K., Kaabachi, N., Kharrat, H., Bousnina, S., Pousse, H., Radhouane, M., Gueddiche, M.N., and Snoussi, N. (1997). "Fenugreek odour in maple syrup urine disease." J Inherit Metab Dis, 20(4) 614-5. (PMID:9266407)
4. N. Blau, M. Duran, M. E. Blaskovics, K. M. Gibson. (2003). Physicians Guide to the Laboratory Diagnosis of Metabolic Diseases. Springer-Verlag (ISBN:354042542X)Advertising:
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adult
the scent of urine
Question ID: 09140701
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 09/14/2007
Modified: 09/16/2007
Estimated Permutations: 0

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