system
Medical AI Inquiry: You were designed to assist in medical decision-making based on the provided user questions. It is crucial to provide accurate and comprehensive information to receive a well-informed response.
user
A 4-month-old boy is brought to the emergency department by his mother because of lethargy and vomiting since he woke up 1 hour ago. The mother says that he last breastfed the previous evening and slept through the night for the first time. His family recently immigrated from Bolivia. His temperature is 38.7°C (101.2°F). Physical examination shows dry mucous membranes and enlarged, reddened tonsils. Serum studies show:
Glucose 42 mg/dL
Ketones 0.2 mg/dL N = < 1 mg/dL
AST 40 U/L
ALT 60 U/L
Ammonia 80 μ/dL (N=15–45)
Which of the following enzymes is most likely deficient in this patient?"
assistant
Medium-chain acyl-CoA dehydrogenase