An infant has hepatorenomegaly, hypoglycemia hyperlipidemia, aciodsis& normal structured glycogen deposition in liver. The most probable diagnosis is:
A. Hers diseaseB. Von Gerke’s disease
C. Con’s disease
D. Andersons disease
E. Pompes disease
The Answer is. (B) Von Gerke’s disease
In von Gierke’s disease there is growth retardation,
enlarged liver and kidney, hypoglycemia elevated blood lactate, cholesterol,
triglyceride and uric acid.
— In von Gierke’s disease the liver biopsy shows
hepatocytes distended with glycogen and
fat with large prominent lipid
vacuoles. There is little associated fibrosis
Related
MCQ’s
Symptoms
|
Constant hunger and need to eat often
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Easy bruising and nosebleeds
|
|
Fatigue, Irritability
|
Puffy cheeks, thin chest and limbs, and swollen
belly
|
Signs:
Delayed puberty
Enlarged liver, Gout, Inflammatory bowel disease
hypoglycemia
Stunting
MCQ - usually diagnosed before age 1.
