A 12 year old girl with an enlarged liver, a low fasting blood glucose concentra

Question

A 12 year old girl with an enlarged liver, a low fasting blood glucose concentration (2.9 mmol/L) and elevated levels of fatty acids and ketone bodies was admitted to the hospital. Glycogen content of her liver was found to be elevated and hepatic glucose-6-phosphatase activity was found to be deficient (about 10% of normal levels). Diagnosis was a type of glycogen storage disease called Van Gierke disease. The disease/metabolic defect would

a. Increase the rate of hepatic gluconeogenesis, resulting in maintenance of blood glucose
b. Decrease the activity of white muscle fibers to use muscle glycogen during intense exercise
c. Decrease the capacity for hepatic glycogenolysis to maintain blood glucose
d. All of the above

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Explanation / Answer

A 12 year old girl with an enlarged liver, a low fasting blood glucose concentration (2.9 mmol/L) and elevated levels of fatty acids and ketone bodies was admitted to the hospital. Glycogen content of her liver was found to be elevated and hepatic glucose-6-phosphatase activity was found to be deficient (about 10% of normal levels). Diagnosis was a type of glycogen storage disease called Van Gierke disease. The disease/metabolic defect would
a. Increase the rate of hepatic gluconeogenesis, resulting in maintenance of blood glucose
b. Decrease the activity of white muscle fibers to use muscle glycogen during intense exercise
c. Decrease the capacity for hepatic glycogenolysis to maintain blood glucose
d. All of the above

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