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Pediatrics Metabolic disorders 89f5939c

All are liver glycogenosis except

A
Von Girke disease
B
Hers disease
C
Type III glycogenosis
D
Pompes disease
High-Yield Explanation
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

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