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Pediatrics Disorders of Amino acid Metabolism 6785577d

This fair-skinned child may be suffering with which of the following diseases?

A
Von Gierke disease
B
Alkaptonuria
C
Albinism
D
Tyrosinemia
High-Yield 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)

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