This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Inborn Errors of Metabolism, Subject name: Pediatrics
Metabolic abnormalities in which jaundice is seen are all of the following except? 
Select and explain. 
A. Von Gierke disease, B. Galactosemia, C. Tyrosinemia, D. Hereditary fructose intolerance
A: Von Gierke disease
Answer: A: Von Gierke disease 
Explanation: a. Von Gierke disease(Ref: Nelson 20/e p 716)In Von Gierke disease, hepatic carbohydrate metabolism is affected, which is responsible for plasma glucose homeostasis. So, fasting hypoglycemia and hepatomegaly is seen. Despite, marked hepatomegaly, the liver transaminase levels are usually normal or only slightly elevated. Jaundice is usually not seen.