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Paediatric

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Metabolic Diseases

Question
8 out of 9
 

In maple syrup urine disease FeClr test with urine gives. color – (AIIMS Nov 2013)



A Green
B Blue
C Red
D Purple

Ans. B

Diagnosis

a. Is usually suspected because of peculiar odour of maple syrup found in the body fluids - urine, sweat, cerumen etc.

b. Diagnosis is confirmed by amino acid analysis showingmarked elevation in plasma level of leucine, isoleucine, valine and alloisoleucine (a steroisomer of isoleucine not normally found in blood) and depression of alanine.

c. Leucine levels are usually higher than those of other three amino acids.

d. Urine contain high levels of leucine, isoleucine and valine and their respective ketoacids.

e. The keto acids may be detected by adding a few drops 2-4 dinitrophenylhydrazine (DNPH) reagent which produces a yellow precipitate in positive test.

f. Ferric chloride gives navy blue colour with the patients urine.

Metabolic Diseases Flashcard List

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