Lipid Biochemistry USMLE Step 1 Practice Question
A previously healthy 18 month old boy is brought to the emergency department for lethargy and a seizure after a viral gastroenteritis with poor oral intake. Laboratory studies show glucose 32 mg per dL, minimal serum ketones, elevated dicarboxylic acids in urine, and mild hyperammonemia. Which of the following enzyme deficiencies is the most likely cause?
Answer choices
- AHMG CoA reductase
- BMedium chain acyl CoA dehydrogenaseCorrect answer
- CGlucose 6 phosphate dehydrogenase
- DCarnitine palmitoyltransferase I
- EPyruvate kinase
- FLong chain acyl CoA dehydrogenase
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