Which metabolic disorder is characterized by severe fasting hypoglycemia, ketosis, hyperlipidemia, lactic acidosis, and hepatorenomegaly?

The metabolic disorder characterized by severe fasting hypoglycemia, ketosis, hyperlipidemia, lactic acidosis, and hepatorenomegaly is Von Gierke's disease, which is a type of glycogen storage disease caused by a deficiency in glucose-6-phosphatase. This enzyme plays a critical role in gluconeogenesis and glycogenolysis, the processes that allow the liver to release glucose into the bloodstream.

In individuals with Von Gierke's disease, the inability to convert glucose-6-phosphate to glucose leads to an accumulation of glycogen in the liver and kidneys, resulting in hepatomegaly. The decreased glucose availability during fasting leads to hypoglycemia, which stimulates lipolysis and ketogenesis, causing hyperlipidemia and ketosis. Additionally, the accumulation of lactic acid occurs due to increased anaerobic glycolysis when the metabolic pathways that depend on glucose are impaired, leading to lactic acidosis.

Other metabolic disorders listed differ significantly in their manifestations and biochemical features. Pompe's disease primarily affects muscle tissues and is characterized by progressive muscle weakness due to a deficiency in acid alpha-glucosidase but does not present with significant hypoglycemia or lactic acidosis. McArdle