**Core Concept**
The boy's symptoms suggest a disorder of glycogen metabolism, leading to excessive glycogen accumulation in the liver and subsequent hypoglycemia. The presence of ketone bodies, lactic acid, and triglycerides indicates a shift towards fat metabolism, a common adaptation in glycogen storage diseases.
**Why the Correct Answer is Right**
The boy's symptoms are consistent with McArdle's disease (Glycogen storage disease type V), a deficiency of the enzyme myophosphorylase. However, the key feature here is the accumulation of glycogen in the liver, pointing towards Glycogen storage disease type I (GSD I), also known as von Gierke's disease. GSD I is caused by a deficiency of glucose-6-phosphatase, an enzyme crucial for glucose production in the liver. Without functional glucose-6-phosphatase, glucose is not released from the liver, leading to hypoglycemia. The liver's inability to metabolize glycogen also leads to its accumulation. The presence of ketone bodies, lactic acid, and triglycerides indicates an adaptation to fat metabolism due to the liver's impaired glucose production.
**Why Each Wrong Option is Incorrect**
**Option A:** This option might seem plausible, but it does not account for the liver's inability to metabolize glycogen, which is a hallmark of GSD I.
**Option B:** This option is not a glycogen storage disease and does not explain the liver's enlargement or the accumulation of glycogen.
**Option C:** This option is a different glycogen storage disease, but it does not fit the clinical presentation described, particularly the accumulation of glycogen in the liver.
**Clinical Pearl / High-Yield Fact**
Glycogen storage disease type I (GSD I) is a rare genetic disorder that requires lifelong management to prevent hypoglycemia and other complications. It is essential to recognize the symptoms and signs of GSD I to provide timely and appropriate treatment.
**Correct Answer: D. Von Gierke's disease (Glycogen storage disease type I)**
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