Glucose, which is a large source of energy to the body is stored in the body in the form of Glycogen. With the help of special proteins, called enzymes, it is released into the blood. Sugar, in the form of glycogen is stored in the body and before carrying out its function, it is needed to be processed by enzymes. If the enzymes are missing, increasing amount of starch in the liver can cause GSD.
Glycogen Storage Disease is caused by:
Inborn error of metabolism, called genetic GSD.
Intoxication with the alkaloid castanospermine, called as acquired GSD.
Lack of enzymes that control the change of glucose into glycogen or release of glucose from glycogen.
Storage of an abnormal amount of glycogen in liver.
Hypoglycemia (deficiency og glucose in the blood.
GSD Type I:
This type of Glycogen Storage Disease is caused due to the lack or absence of Glucose-6-Phosphates enzyme. It is also called von Gierke disease or hepatorenal glycogenosis. The incident takes place in 1 out of 50.000 births.
GSD Type II:
Lack of acid alpha-glucosidase enzyme causes Glycogen Storage Disease II. It is also known as acid maltase deficiency or Pompe’s disease. 1 out of 40,000 new born babies is captured by this diseases. Death occurs usually at the age of 2 due to heart failure.
GSD Type III:
GSD Type III is also names as Forbes-Cori disease or limit dextrinosis. Glycogen debranching enzyme causes this disorder. 1 infant out of 100,000 is affected. Myopathy is the symptom of GSD type III.
GSD Type IV:
Lack of glycogen branching enzyme becomes the reason of this type of GSD. It is found in 1 child out of 500,000. This is also known as amylopectinosis or Andersen disease. It causes failure to thrive and death occurs at the age of 5 years. There are no muscle symptoms appear in GSD type IV.
GSD Type V:
Another name of GSD type V is McArdle disease. It is caused by the lack of muscle glycogen phosphorylase. 1 out of 100,000 infants is infected by this disorder. Symptom of this disorders include Exercise-induced cramps and renal failure.
GSD Type VI:
This also called hers’ disease. It is found in 1 out of 65,000 new born babies. Liver glycogen phosphorylase enzyme deficiency causes Hers’ disease.
GSD Type VII:
GSD Type VII known as Tarui disease is caused due to the deficiency of muscle phosphofructokinase. It causes growth retardation in the infants. Symptoms include Exercise-induced muscle cramps, weakness and Haemolytic anaemia. 1 infant out of 1,000,000 is affected.
GSD Type VIII:
Deficiency of phosphorylase kinase and PHKA2 enzymes is the reason of GSD Type VIII. This order causes delayed development and growth retardation.
GSD Type IX:
This is also known as Fanconi-Bickel syndrome and is caused due to the lack of glucose transporter, GLUT2 enzyme.
GSD Type X:
This type of GSD is called as Red cell aldolase deficiency. It is caused because of the deficiency of Aldolase A enzyme. It increases the intensity of myalgias over decades. Symptoms include cramps, exercise intolerance and episodic elevations.
GSD Type 0:
Deficiency of glycogen synthase enzyme causes GSD type 0. Its symptom is occasional muscle cramping. In some cases, it causes death.
Treatments of Glycogen Storage Disease (GSD):
Some of the Glycogen Storage Diseases cannot be controlled while other can be cured easily. Treatment depends on the type of GSD. Following are the ways to treat Glycogen Storage Disease:
Night time feeding:
Children need a feeding tube into their stomach for keeping the glucose level balanced. It is use to give a high quantity of glucose which helps in controlling blood sugar level.
Giving uncooked cornstarch every four to six hours to the child may prove helpful in controlling the blood sugar level
Meals enriched with carbohydrates many times a day:
Several small means packed with carbohydrates help preventing blood sugar level from decreasing.
Medication is compulsory because GSD causes production of uric acid in the body of the child which may cause join inflammation is the body which is quite painful.