the scent of urine

Topic: Adult

Created on Friday, September 14 2007 by jdmiles

Last modified on Sunday, September 16 2007.

 
        A) Tyrosinemia type 1 is associated with a "rancid butter" smell.
 
        B) Methionine malabsorption is associated with a "cabbage" smell.
 
        C) Methylmalonic acidemia is associated with an "acid" smell.
 
        D) Phenylketonuria is associated with a "fenugreek" smell.
 
        E) Maple syrup urine disease is associated with a "maple syrup" 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) tyrosinemia type 1 is associated with a "rancid butter" smell.

This answer is incorrect.

The statement, "tyrosinemia type 1 is associated with a "rancid butter" smell." is true.
tyrosinemia type 1 is associated with a "cabbage" smell, and is associated with a "rancid butter" smell.  (See References)

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

This answer is incorrect.

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

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C) methylmalonic acidemia is associated with an "acid" smell.

This answer is incorrect.

The statement, "methylmalonic acidemia is associated with an "acid" smell." is true.
methylmalonic acidemia is associated with an "acid" smell.  (See References)

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D) phenylketonuria is associated with a "fenugreek" smell.

This answer is correct.

The statement, "phenylketonuria is associated with a "fenugreek" 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 "maple syrup" smell.

This answer is incorrect.

The statement, "maple syrup urine disease is associated with a "maple syrup" 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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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