the scent of urine

Topic: Adult

Created on Friday, September 14 2007 by jdmiles

Last modified on Sunday, September 16 2007.

 
        A) Methylmalonic acidemia is associated with a "burnt sugar" smell.
 
        B) Methionine malabsorption is associated with a "sulfur" smell.
 
        C) Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "sulfur" smell.
 
        D) 3-hydroxy-3-methylglutaric aciduria is associated with a "sweaty feet" smell.
 
        E) Isovaleric acidemia is associated with a "fenugreek" 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

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A) methylmalonic acidemia is associated with a "burnt sugar" smell.

This answer is incorrect.

The statement, "methylmalonic acidemia is associated with a "burnt sugar" smell." is false.
methylmalonic acidemia is associated with an "acid" smell.  (See References)

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B) methionine malabsorption is associated with a "sulfur" smell.

This answer is incorrect.

The statement, "methionine malabsorption is associated with a "sulfur" smell." is false.
methionine malabsorption is associated with a "cabbage" smell.  (See References)

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C) multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "sulfur" smell.

This answer is incorrect.

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

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D) 3-hydroxy-3-methylglutaric aciduria is associated with a "sweaty feet" smell.

This answer is correct.

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

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E) isovaleric acidemia is associated with a "fenugreek" smell.

This answer is incorrect.

The statement, "isovaleric acidemia is associated with a "fenugreek" smell." is false.
isovaleric acidemia is associated with a "sweaty feet" 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