This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Disorders of Amino acid Metabolism, Subject name: Pediatrics
This fair-skinned child may be suffering with which of the following diseases? 
Select and explain. 
A. Von Gierke disease, B. Alkaptonuria, C. Albinism, D. Tyrosinemia
C: Albinism
Answer: C: Albinism 
Explanation: Defect in Tyrosinase leads to Albinism, which presents with hypopigmentation. Von Gierke disease- doll like facies Alkaptonuria- grey discoloration of sclera, ear & nose cailage (ochronosis)