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Question 127 / 280
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CLINICALPATHOLOGY-92
MAPLE SYRUP URINE DISEASE (MSUD) is caused by deficiency of which enzyme and how is it detected on urine testing?
A. Phenylalanine hydroxylase deficiency; ferric chloride gives olive green
B. BRANCHED-CHAIN ALPHA-KETO ACID DECARBOXYLASE (BCKD) deficiency; maple syrup urine odor + ferric chloride gives GREY-GREEN; leucine, isoleucine, valine in urine
C. Galactose-1-phosphate uridyltransferase deficiency; Benedict's test positive
D. Homogentisate oxidase deficiency; urine darkens on standing
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