Liver Glycogenoses
Disorders with hepatomegaly and hypoglycemia
- Type I-Von Gierke -Glucose 6 phosphatase
- Type III-Cori/Forbe limit dextrinosis- Debranching enzyme deficiency
- Type VI-Her’s Liver phosphorylase
- Type IX-Phosphorylase Kinase deficiency
- Type XI-Fancon/Bickel Glut 2 deficiency
With Liver cirrhosis
Type IV-Anderson’s -Branching enzyme deficiency
Muscle Glycogenoses
With muscle energy impairment
- Type V- Mc Ardle – muscle phosphorylase
- Type VII-Tarui-Phosphofructokinase deficiency
With progressive skeletal muscle myopathy/cardiomyopathy
Type II-Pompe’s Lysosomal acid Alpha glucosidase deficiency
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