Glycogen Storage diseases

Liver Glycogenoses

Disorders with hepatomegaly and hypoglycemia

  1. Type I-Von Gierke -Glucose 6 phosphatase
  2. Type III-Cori/Forbe limit dextrinosis- Debranching enzyme deficiency
  3. Type VI-Her’s Liver phosphorylase
  4. Type IX-Phosphorylase Kinase deficiency
  5. Type XI-Fancon/Bickel Glut 2 deficiency

With Liver cirrhosis

Type IV-Anderson’s -Branching enzyme deficiency

Muscle Glycogenoses

With muscle energy impairment

  1. Type V- Mc Ardle – muscle phosphorylase
  2. Type VII-Tarui-Phosphofructokinase deficiency

With progressive skeletal muscle myopathy/cardiomyopathy

Type II-Pompe’s Lysosomal acid Alpha glucosidase deficiency

  1. Glycogen storage disorders mnemonic | Master Of Medicine - pingback on February 25, 2011 at 5:30 am

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