Inborn Errors of Metabolism · USMLE Step 1

A child with episodic hypoglycemia and accumulation of fatty-acid intermediates during fasting suggests a disorder in which metabolic pathway?

  1. Amino acid catabolism
  2. Fatty acid β-oxidation
  3. Glycolysis
  4. Urea cycle
Show answer and explanation

Correct answer: Fatty acid β-oxidation

Fatty-acid β-oxidation disorders impair the body’s ability to generate energy from fats during fasting, leading to hypoglycemia and buildup of fatty-acid intermediates.

Difficulty: Medium Question 6 of 19

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