An 8-Year-Old Boy Is Brought to the Emergency Department Due

question 101

Multiple Choice

An 8-year-old boy is brought to the emergency department due to vomiting and lethargy.  The patient had been on an overnight hiking trip with his family.  During the trip, the family lost their food pack while canoeing and had to hike back to their car.  The child became weak and was carried for the last mile.  None of the family has eaten for approximately 24 hours.  On examination, the patient appears listless.  Mild hepatomegaly is noted.  Laboratory results are as follows: The patient begins seizing shortly after arriving at the emergency department.  Which of the following enzymes is most likely deficient in this patient?

• A)Acetyl-CoA carboxylase
• B)Acid alpha-glucosidase
• C)Acyl-CoA dehydrogenase
• D)Glucose 6-phosphatase
• E)Glycogen phosphorylase

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