the scent of urine

Topic: Adult

Created on Friday, September 14 2007 by jdmiles

Last modified on Sunday, September 16 2007.

 
        A) Multiple carboxylase deficiency is associated with a "curry" smell.
 
        B) Methylmalonic acidemia is associated with a "maple syrup" smell.
 
        C) Trimethylaminuria is associated with an "acid" smell.
 
        D) Maple syrup urine disease is associated with a "burnt sugar" smell.
 
        E) Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "fish market" 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) multiple carboxylase deficiency is associated with a "curry" smell.

This answer is incorrect.

The statement, "multiple carboxylase deficiency is associated with a "curry" smell." is false.
multiple carboxylase deficiency is associated with a "cat urine" smell.  (See References)

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B) methylmalonic acidemia is associated with a "maple syrup" smell.

This answer is incorrect.

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

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C) trimethylaminuria is associated with an "acid" smell.

This answer is incorrect.

The statement, "trimethylaminuria is associated with an "acid" smell." is false.
trimethylaminuria is associated with a "fish market" smell.  (See References)

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D) maple syrup urine disease is associated with a "burnt sugar" smell.

This answer is correct.

The statement, "maple syrup urine disease is associated with a "burnt sugar" smell." is true.
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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E) multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "fish market" smell.

This answer is incorrect.

The statement, "multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type 2) is associated with a "fish market" 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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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