Factors responsible for ketosis in a patient of von Gierke&;s disease are all, except
## Core Concept
Von Gierke's disease, also known as glycogen storage disease type I (GSD I), is a genetic disorder caused by deficiency of the enzyme glucose-6-phosphatase. This enzyme deficiency leads to an inability to convert glucose-6-phosphate into glucose for release into the bloodstream, resulting in severe hypoglycemia, hepatomegaly, and lactic acidosis. Ketosis in this context arises due to the body's need to find alternative sources of energy.
## Why the Correct Answer is Right
The correct answer, **D. Increased insulin level**, is right because in von Gierke's disease, the primary issue is the inability to produce glucose from glycogen or gluconeogenesis. This leads to hypoglycemia. The body compensates for the lack of glucose by breaking down fats to produce ketones, a process that is enhanced by low insulin levels. Insulin inhibits ketogenesis; therefore, increased insulin levels would not contribute to ketosis but rather prevent it.
## Why Each Wrong Option is Incorrect
- **Option A: Decreased glucose level** - This is a contributing factor to ketosis. When glucose levels are low, the body begins to break down fat for energy, leading to the production of ketones.
- **Option B: Increased glucagon level** - Glucagon promotes gluconeogenesis and glycogenolysis, but in the context of von Gierke's disease, these pathways are impaired. However, the increased glucagon level does contribute to the metabolic state that leads to ketosis by signaling the need for alternative energy sources.
- **Option C: Increased epinephrine level** - Like glucagon, epinephrine (adrenaline) prepares the body for 'fight or flight' by increasing glucose levels in the blood. It does so by promoting glycogenolysis and gluconeogenesis. In von Gierke's disease, despite these efforts, glucose levels remain low, and the body resorts to ketogenesis.
## Clinical Pearl / High-Yield Fact
A key point to remember is that patients with von Gierke's disease are prone to ketosis due to their inability to maintain normoglycemia. This condition leads to a clinical picture that includes not only hypoglycemia but also lactic acidosis, hepatomegaly, and hyperlipidemia. The development of ketosis is a critical adaptation but also a clue to the underlying metabolic defect.
## Correct Answer: D. Increased insulin level