<prompt> This fair-skinned child may be suffering with which of the following diseases?Von Gierke diseaseAlkaptonuriaAlbinismTyrosinemia  <response>: 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 