A 3-year-old girl is brought to the emergency department by her parents for vomiting and lethargy for the past day and a new seizure one hour ago that lasted approximately 3 minutes. They report that she is taking amoxicillin for otitis media that was diagnosed two days ago. On physical examination, she is noted to have a fruity odor and has dry mucous membranes. A urine sample is obtained and is noted to have a distinct sweet-smelling odor. Laboratory results confirm a diagnosis of maple syrup urine disease (MSUD).
Which of the following amino acids is least likely to be elevated in this patient?
- A) Cysteine
- B) Isoleucine
- C) Leucine
- D) Valine
Author(s)
Dr. Ted O'Connell
Matthew Christensen
Editor(s)
Dr. Ted O'Connell
Last updated
May 10, 2022
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