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Glycogen Storage Disease Type I Summary

Description: An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.

Also Known As: Deficiency, Glucosephosphatase; Gierke Disease; Gierke's Disease; Glycogen storage disease 1 (GSD I); von Gierke's Disease Show All >>

Networked: 166 relevant articles (2 outcomes, 14 trials/studies) for this Disease

Key Drugs and Agents for Glycogen Storage Disease Type I

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Glucose (Dextrose) : 1 outcome in 27 results : FDA 175 Generic
  2. Glycogen : 3 studies in 20 results : IBA
  3. Glucose-6-Phosphatase (Glucose 6 Phosphatase) : 2 studies in 40 results : IBA
  4. Glucagon (Glukagon) : 2 studies in 6 results : FDA 4
  5. Liver Glycogen : 1 study in 5 results : IBA
  6. Lactic Acid : 1 study in 4 results : FDA 4 Generic
  7. Insulin (Novolin) : 1 study in 4 results : FDA 2
  8. Uric Acid (Urate) : 1 study in 3 results : IBA
  9. purine : 1 study in 3 results : IBA
  10. Leucine (L-Leucine) : 1 study in 1 result : FDA 31
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Diseases Related to Glycogen Storage Disease Type I

  1. Hypoglycemia (Reactive Hypoglycemia)
  2. Glycogen Storage Disease Type IV (Andersen's Disease)
  3. Glycogen Storage Disease (Glycogenosis)
  4. Hyperlipidemias (Hyperlipidemia)
  5. Proteinuria
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Key Therapies for Glycogen Storage Disease Type I

Efficacy Chart >>
  1. Continuous Ambulatory Peritoneal Dialysis (CAPD) : 1 outcome in 2 results
  2. Liver Transplantation : 1 study in 4 results
  3. Pancreatectomy : 1 study in 1 result
  4. Kidney Transplantation : 4 results
  5. Transplants (Transplant) : 1 result
Show All >>

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