Glycogen Storage Disease Type VII Causes, Symptoms and Treatment and Related Disorders ...
Glycogen Storage Diseases
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Glycogen Storage Diseases
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What is glycogen storage disease type I?
Glycogen storage disease type I (also known as GSDI or von Gierke disease) is an inherited disorder caused by the buildup of a complex sugar called glycogen in the body's cells.
Glycogen storage diseases (GSDs) are a group of inherited genetic disorders. They cause glycogen to be improperly formed or released in the body. This results in a build up of abnormal amounts or types of glycogen in tissues.
A hepatic glycogen storage disease in which there is an apparent deficiency of hepatic phosphorylase activity.
Glycogen storage disease type 2 (GSD II) is a lysosomal storage disease which particularly affects the skeletal and respiratory muscles with varying degrees of gravity and which, in the infantile form, is associated with hypertrophic cardiomyopathy.
Alternate Names : Glycogen storage disease type V (GSDV), Myophosphorylase deficiency, Muscle glycogen phosphorylase deficiency, PGYM deficiency
Definition ...
Glycogen storage diseases
A group of birth defects that change the way the liver breaks down glycogen. See also Glycogen.
Glycogen storage disease: Any one of the 14 recognized diseases that interfere with the storage of carbohydrates as glycogen in the body, aka McArdle's disease.
Glycogen Storage Disease Type II
General Discussion
Pompe disease is a glycogen storage disease.
Glycogen storage diseases or glycogenoses are a group of disorders due to a lack of one or more enzymes needed to break down glucose into its storage form, glycogen, and back from glycogen to glucose when the body requires it.
Glycogen storage disease type V (GSDV); Myophosphorylase deficiency; Muscle glycogen phosphorylase deficiency; PGYM deficiency
Causes ...
Glycogen storage diseases are caused by deficiencies of enzymes involved in glycogen synthesis or breakdown; ...
Glycogen storage diseases
Infections, such as hepatitis A, B, and C
Liver cancer and benign liver tumors ...
Glycogen Storage Disease Type I ... joint disease
Gonorrhea ... gonococcal arthritis
Gout ... arthritis, joint immobility
Group A Streptococcal Infections ... joint infections (septic arthritis) ...
Glycogen storage disease
Any of a number of rare inborn errors of metabolism caused by defects in specific enzymes or transporters involved in the metabolism of glycogen
Glycogen ...
McArdle syndrome (glycogen storage disease type V)
McCune-Albright syndrome
MD
Measles
Meatal stenosis
Meckel's diverticulum
Meconium aspiration (inhalation)
Meconium aspiration syndrome (MAS)
Median nerve dysfunction
Mediastinal emphysema ...
Glucose-6-Phosphatase Deficiency Glycogen Storage Disease
Glucose-6-Phosphate Dehydrogenase Deficiency
Glucose-Galactose Malabsorption
Glucose-Galactose Malabsorption
Glucosyl cerebroside lipidosis
Glucosylceramidase deficiency ...
Lactose intolerance - Glycogen storage disease (type I, type II, type III, type IV, type V, type VI, type VII) - fructose metabolism (Fructose intolerance, Fructose bisphosphatase deficiency, Essential fructosuria) - galactose metabolism ...
Cori disease see Glycogen Storage diseases
Cornelia de Lange syndrome
Coronal Synostosis see Craniofacial conditions
Cortical Malformations
Corticobasal Degeneration
Costello syndrome
Cot Death
Cowden disease
Cowden syndrome see Cowden disease ...
Acid maltase deficiency is also known as glycogen storage disease type II (GSD II) because it is characterized by a buildup of glycogen in the muscle cells. Glycogen is the chemical substance muscles use to store sugars and starches for later use.
McArdle disease: Glycogen storage disease type V and the most common type of glycogen storage disease.
Visser G, Rake JP, Labrune P, et al; Consensus guidelines for management of glycogen storage disease type 1b - European Study on Glycogen Storage Disease Type 1. Eur J Pediatr. 2002 Oct;161 Suppl 1:S120-3. Epub 2002 Sep 13. [abstract] ...
[6] Other risk factors include alcoholic cirrhosis, hemochromatosis, alpha-l-antitrypsin deficiency, glycogen storage disease, porphyria cutanea tarda, tyrosinemia, and Wilson disease,[2] but rarely biliary cirrhosis.
type I glycogen storage disease, which causes problems in controlling blood sugars when a person fasts
porphyria, a condition that causes a malfunction in how the body uses porphyrins.
other myopathiesincluding congenital myopathies, glycogen storage diseases and other metabolic myopathies, polymyositis and dermatomyositis ...
Metabolic disorders, such as thyroid disease and glycogen storage disease. (In glycogen storage disease, the body's cells don't use a form of glucose (sugar) properly.) ...
Other, less common causes of liver cancer include glycogen storage disease, anabolic steroids, accumulation of copper in the liver due to a genetic disorder (Wilson's disease), ...
glycogen storage diseases of muscle: caused by mutations in genes controlling enzymes that metabolize glycogen and glucose (blood sugar); include Pompe's, Andersen's and Cori's diseases ...
Cystic fibrosis, alpha-1 antitrypsin deficiency, hemochromatosis, Wilson disease, galactosemia, and glycogen storage diseases are inherited diseases that interfere with how the liver produces, processes, and stores enzymes, proteins, metals, ...
For instance, cystinosis galactosemia, glycogen storage disease, hereditary fructose intolerance, Lowe syndrome, Wilson disease, tyrosinemia, medullary cystic disease, and vitamin D dependency, have been shown to cause Fanconi syndrome.
Von Gierke disease is also called Type I glycogen storage disease (GSD I).
Alternative Names
Type I glycogen storage disease ...
Synonyms: brancher glycogen storage disease
The information shown above for debrancher deficiency is provided by Stedman's.
Inherited diseases including Alpha-1 antitrypsin deficiency, hemochromatosis, Wilson's disease, galactosemia, and glycogen storage diseases interfere with the way the liver produces, processes, and stores enzymes, proteins, metals, ...
Metabolic diseases: These include glycogen storage diseases. Variable neutrophil counts are observed.
An inherited liver disease, such as Wilson's disease, hemochromatosis, alpha1-antitrypsin deficiency, glycogen storage disease, and autoimmune hepatitis
Prolonged exposure to certain toxins
Certain forms of heart disease ...
Storage diseases such as hemochromatosis and glycogen storage disease
Heart disease
Muscle disease
Malnutrition
Alcoholism
Radiation exposure
Exposure to certain drugs (eg, lithium, sulfonamides, cancer chemotherapy , phenothiazines) ...
They include Wilson disease, cystic fibrosis, alpha-1 antitrypsin deficiency, hemochromatosis, galactosemia, and glycogen storage disease.
Most of these diseases are not common but they can be devastating.
These include diseases that cause excess copper or iron to be deposited in the liver as well as galactosemia - a rare disorder that affects the way the body metabolizes milk sugar (lactose) - and glycogen storage diseases, which prevent glycogen, ...
Glycogen storagedisease (GSD) involves defects that cause an abnormal accumulation of glycogen, usually found in the liver, muscle, or both. When accumulation occurs in the liver, glycogen storage diseases result in liver enlargement and in ...
Maternal hyperglycemia due to diabetes
Prolonged fasting
Congenital hyperinsulinism several types, both transient and persistent
Inborn errors of carbohydrate metabolism such as glycogen storage disease
Insulin-induced hypoglycemia ...
Other genetic conditions associated with liver cancer include several inborn errors of metabolism such as tyrosinemia, glycogen storage disease type I, galactosemia, and alpha1-antitrypsin deficiency.
Inherited metabolic disorders that increase the risk of cirrhosis include galactosemia, a rare disorder that affects the way the body metabolizes milk sugar (lactose), and glycogen storage diseases, which prevent glycogen, the stored form of glucose, ...
 See also: Storage disease, Deficiency, Symptom, Cancer, Kidney
  
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