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Glycogen Storage Disease Type I

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; Deficiencies, Glucose-6-Phosphatase; Deficiencies, Glucosephosphatase; Deficiency, Glucose-6-Phosphatase; Disease, Gierke; Disease, Gierke's; Disease, von Gierke; Disease, von Gierke's; Gierkes Disease; Glucose 6 Phosphatase Deficiency; Glucose-6-Phosphatase Deficiencies; Glucosephosphatase Deficiencies; von Gierkes Disease; Glucose-6-Phosphatase Deficiency; Glucosephosphatase Deficiency; Glycogenosis 1; Hepatorenal Glycogen Storage Disease; von Gierke Disease
Networked: 166 relevant articles (2 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1. Hasanoğlu, Alev: 3 articles (01/2013 - 01/2012)
2. Tümer, Leyla: 3 articles (01/2013 - 01/2012)
3. Koeberl, Dwight D: 3 articles (07/2012 - 07/2007)
4. Melis, Daniela: 2 articles (04/2015 - 04/2008)
5. Parini, Rossella: 2 articles (04/2015 - 05/2007)
6. Parenti, Giancarlo: 2 articles (04/2015 - 04/2008)
7. Austin, Stephanie L: 2 articles (12/2013 - 04/2010)
8. Kishnani, Priya S: 2 articles (12/2013 - 04/2010)
9. Kasapkara, Çiğdem Seher: 2 articles (01/2013 - 01/2013)
10. Tashiro, Hirotaka: 2 articles (06/2012 - 01/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Glycogen Storage Disease Type I:
1. Glucose (Dextrose)FDA LinkGeneric
2. GlycogenIBA
3. Glucose-6-Phosphatase (Glucose 6 Phosphatase)IBA
4. Glucagon (Glukagon)FDA Link
5. Liver GlycogenIBA
6. Lactic AcidFDA LinkGeneric
7. Insulin (Novolin)FDA Link
8. Uric Acid (Urate)IBA
9. purineIBA
10. Leucine (L-Leucine)FDA Link

Therapies and Procedures

1. Continuous Ambulatory Peritoneal Dialysis (CAPD)
2. Liver Transplantation
3. Pancreatectomy
4. Kidney Transplantation
5. Transplants (Transplant)