Quiz Form

Question Date: 02/09/2023

Question: 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

Options:

• Non Branched-chain amino acidfree and low protein diets
• Branched-chain amino acidfree formulas and low-protein diets
• Non Branched-chain amino acidfree formulas and low-carbohydrate diets
• Branched-chain amino acidfree formulas and low-carbohydrate diets

Correct Answer: Branched-chain amino acidfree formulas and low-protein diets

Explaination: Patient has maple syrup urine disease and hence is advised Branched-chain amino acidfree formulas and low-protein diets

Reference: Patient has maple syrup urine disease and hence is advised Branched-chain amino acidfree formulas and low-protein diets