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Glycogen Storage Disease Type III (Cori's Disease) Summary

Description: An autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). The clinical course of the disease is similar to that of glycogen storage disease type I, but milder. Massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. Levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. Six subgroups have been identified, with subgroups Type IIIa and Type IIIb being the most prevalent.

Also Known As: Cori's Disease; Forbes Disease; Cori Disease; Deficiency, Debrancher; Glycogen Debrancher Deficiency Show All >>

Networked: 87 relevant articles (1 outcomes, 3 trials/studies) for this Disease

Key Drugs and Agents for Glycogen Storage Disease Type III

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Glucose (Dextrose) : 1 outcome in 5 results : FDA 175 Generic
  2. amylo-1,6-glucosidase : 2 studies in 11 results : IBA
  3. Glycogen Debranching Enzyme System (Glycogen Debranching Enzyme) : 40 results : IBA
  4. Glycogen : 31 results : IBA
  5. Glucosidases : 5 results : IBA
  6. Transferases : 3 results : IBA
  7. alpha-Glucosidases (Acid Maltase) : 3 results : IBA
  8. caloreen : 3 results : IBA
  9. Starch (Cornstarch) : 2 results : IBA
  10. Transaminases : 2 results : IBA
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Diseases Related to Glycogen Storage Disease Type III

  1. Glycogen Storage Disease (Glycogenosis)
  2. Glycogen Storage Disease Type I
  3. Glycogen Storage Disease Type IV (Andersen's Disease)
  4. Liver Cell Adenoma (Hepatocellular Adenoma)
  5. Fibrosis (Cirrhosis)
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Key Therapies for Glycogen Storage Disease Type III

Efficacy Chart >>
  1. Liver Transplantation : 1 study in 1 result
  2. Enzyme Replacement Therapy : 1 result
  3. Carbohydrate-Restricted Diet (Low Carbohydrate Diet) : 1 result
  4. Cardiopulmonary Bypass (Heart-Lung Bypass) : 1 result

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