All of the following are true about von Gierke&;s disease except
Glycogen storage disease type I (GSD I) or von Gierke&;s disease, is the most common of the glycogen storage diseases. This genetic disease results from deficiency of the enzyme glucose-6-phosphatase and has an incidence in the American population of approximately 1 in 50,000 to 100,000 bihs. The deficiency impairs the ability of the liver to produce free glucose from glycogen and from gluconeogenesis. Since these are the two principal metabolic mechanisms by which the liver supplies glucose to the rest of the body during periods of fasting, it causes severe hypoglycemia and results in increased glycogen storage in liver and kidneys. Both organs function normally in childhood but are susceptible to a variety of problems in adult years. Other metabolic derangements include lactic acidosis and hyperlipidemia. Frequent or continuous feedings of cornstarch or other carbohydrates are the principal treatment. Other therapeutic measures may be needed for associated problems.Ref: DM Vasudevan, 7th edition, page no: 128
Metabolism of carbohydrate
G-6 Phosphatase deficiency
Hypoglycemia unresponsive to epinephrine but not glucagon
Glycogen accumulates in kidney and liver
Hyperglycemia
Hyperglycemia