All are liver glycogenosis except: (A) Von Girke disease, (B) Hers disease, (C) Type III glycogenosis, (D) Pompes disease
Answer is D. Pompe disease is a muscle glycogenoses(Type II)due to enzymatic defect of Lysosomal alpha 1,4-glucosidase(acid maltase). Von Gierke is due to defect of glucose-6-phosphatase(Type Ia).Type III glycogenoses are Cori/Forbes(IIIa-liver and muscle debrancher deficiency) & Type IIIb.Hers is Type VI glycogenoses due to enzymatic defect of liver phosphorylase. Reference:Essential pediatrics-Ghai,8th edition,page no:657