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A 6 year old girl was brought to the emergency department with fever, vomiting, altered sensorium. Her MRI Brain and lumbar puncture was normal.<br><br> Electrolytes, renal and liver function tests were normal. She had metabolic acidosis and a characteristic burnt sugar odor. Her serum amino acids showed elevated levels of leucine, isoleucine, valine, and their keto acid derivatives.<br>After supportive aggressive acute therapy what will be your diet prescription for her
Explanation
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Patient has maple syrup urine disease and hence is advised Branched-chain amino acidfree formulas and low-protein diets
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Reference
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Patient has maple syrup urine disease and hence is advised Branched-chain amino acidfree formulas and low-protein diets
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Option 1
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Is Correct (Option 1):
Non Branched-chain amino acidfree and low protein diets
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Option 2
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Is Correct (Option 2):
Branched-chain amino acidfree formulas and low-protein diets
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Option 3
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Is Correct (Option 3):
Non Branched-chain amino acidfree formulas and low-carbohydrate diets
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Option 4
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Is Correct (Option 4):
Branched-chain amino acidfree formulas and low-carbohydrate diets
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