Metabolism USMLE Step 1 Practice Question
A 3-year-old female who is small for her age has had multiple hypoglycemic episodes associated with moderate fasting during periods of illness. In her most recent episode associated with influenza, her parents slept late, and at 10 AM were unable to rouse the child from sleep. Blood and urine samples collected at the emergency department reveal marked hypoglycemia, ketonuria, and ketonemia along with an appropriately low insulin level. The blood alanine level is abnormally low; however, infusion of alanine produces a rapid rise in blood glucose. The defect most likely responsible for these symptoms is found in which of the following pathways?
Answer choices
- AGlycogenolysis
- Bβ-Oxidation of fatty acids
- CGluconeogenesis
- DTriglyceride hydrolysis in adipose tissue
- EProtein catabolism in muscleCorrect answer
- FFructose-1,6-bisphosphatase activity
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