Inborn Errors of Metabolism · USMLE Step 1

A patient with episodic metabolic decompensation, vomiting, acidosis, and elevated organic acids in urine likely has which class of IEM?

  1. Amino acidopathy
  2. Organic acidemia / aciduria
  3. Glycogen storage disease
  4. Urea cycle defect
Show answer and explanation

Correct answer: Organic acidemia / aciduria

Organic acidemias (or acidurias) lead to accumulation of toxic organic acids, causing metabolic acidosis, vomiting, and metabolic decompensation, especially during catabolic stress.

Difficulty: Medium Question 14 of 19

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