Inborn Errors of Metabolism · USMLE Step 1

A newborn with elevated blood galactose after feeding develops jaundice, vomiting and liver dysfunction. This scenario best fits which IEM category?

  1. Amino acidopathy of branched-chain amino acids
  2. Carbohydrate (galactose) metabolism disorder
  3. Mitochondrial fatty acid oxidation disorder
  4. Urea cycle disorder with hyperammonemia
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Correct answer: Carbohydrate (galactose) metabolism disorder

Galactosemia is a carbohydrate-metabolism inborn error; inability to metabolize galactose leads to accumulation of toxic metabolites causing liver dysfunction, jaundice, and failure to thrive in neonates.

Difficulty: Medium Question 19 of 19

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