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Glycogen Storage Disease Type IV (Andersen's Disease) Summary

Description: An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal GLYCOGEN with long outer branches. Clinical features are MUSCLE HYPOTONIA and CIRRHOSIS. Death from liver disease usually occurs before age 2.

Also Known As: Andersen's Disease; Glycogenosis, Type IV; Amylopectinosis; Andersen Disease; Deficiency, Brancher Show All >>

Networked: 80 relevant articles (0 outcomes, 3 trials/studies) for this Disease

Key Drugs and Agents for Glycogen Storage Disease Type IV

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Glycogen : 1 study in 13 results : IBA
  2. chitotriosidase : 1 study in 3 results : IBA
  3. Neuraminidase deficiency with beta-galactosidase deficiency : 1 study in 1 result : IBA
  4. 1,4-alpha-Glucan Branching Enzyme (Glycogen Branching Enzyme) : 40 results : IBA
  5. polyglucosan : 22 results : IBA
  6. Amylopectin : 12 results : IBA
  7. Fibrinogen (Factor I) : 2 results : FDA 1
  8. Cyanamide (Calcium Carbimide) : 2 results : IBA
  9. 1,4-glucan : 2 results : IBA
  10. Nonsense Codon (Nonsense Mutation) : 1 result : IBA
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Diseases Related to Glycogen Storage Disease Type IV

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

Efficacy Chart >>
  1. Liver Transplantation : 1 study in 5 results
  2. Transplants (Transplant) : 2 results
  3. Heart Transplantation (Grafting, Heart) : 1 result
  4. Transplantation (Transplant Recipients) : 1 result

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