Treating pyruvate dehydrogenase deficiency
A child with pyruvate dehydrogenase deficiency has lactic acidosis. Which dietary change would best supply acetyl-CoA without worsening the acidosis?
Answer C: explanation for every choice
A
More carbohydrate makes more pyruvate, which is shunted to lactate.
B
Alanine is converted to pyruvate, adding to the backlog.
C
Correct. Fatty acids and ketogenic amino acids produce acetyl-CoA directly, bypassing pyruvate dehydrogenase.
D
Glucose also produces pyruvate and would worsen lactic acidosis.
TakeawayBypass a pyruvate dehydrogenase block with fuels that make acetyl-CoA without pyruvate.
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Reviewed 2026-11-05 by a medical student. Spot an error? Report it with question ID bb158.