Phenylketonuria – Aminoacids – Biochemistry

Phenylketonuria?
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Phenylalanine hydroxylase

Ferric chloride test in phenylketonuria gives?
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Green colour

Phenylketonuria is diagnosed by?
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Guthrie test, Ferric chloride test

Phenylpyruvic acid in urine is detected by?
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Ferric chloride test

NOT true about Phenylketonuria?
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50% ECG changes

Phenylketonuria?
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NOT true about treatment of phenyketonuria?
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Phenylalanine should be completely stopped in diet

First line of therapy in phenylketonuria?
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Limiting the substrate for deficient enzyme

In phenylketonuria, the main aim for first line therapy?
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Limiting the substrate for deficient enzyme

NOT a feature of Phenylketonuria?
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Reduced tendon reflex
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