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ervices Hillman Center for Pediatric Transplantation Pediatric Liver Transplant Educational Resources Liver Disease States Storage
Glycogen " Diseases
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Liver Transplant
Glycogen Storage Diseases (GSD) in Children
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What Is Glycogen Storage Disease? Doctor
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Glycogen storage diseases (GSDs) are a group of inherited genetic disorders
that cause glycogen to be improperly stored in the body. Children with
What to Expect During Locations &
Liver Transplant glycogen storage diseases have a buildup of abnormal amounts or types of Directions
glycogen in their tissues.
Recovery After Liver
Transplant Glycogen is the storage form of glucose in our bodies. Glucose is a simple Contact Us
sugar, which is a form of carbohydrate. It is found in many foods and is the
Living-Donor Liver main source of energy in our bodies.
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Types of Glycogen Storage Disease
Metabolic Disease
The main types of glycogen storage diseases in children are categorized by
Our Doctors & Staff number and name. They include: Pay My Bill
Type I (Von Gierke disease) – this is the most common type of glycogen
Patient & Family
Resources storage disease, and accounts for 90% of all glycogen storage disease
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cases
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Type II (Pompe's disease, acid maltase deficiency)
Organs Since glycogen is primarily stored in the liver or muscle tissue, glycogen
Liver Transplant Patient storage diseases usually affect functioning of the liver, the muscles, or both.
Procedures The glycogen storage diseases that mainly affect the liver are types I, III, IV, and
Liver Transplant Tests VI. The glycogen storage diseases that mainly affect muscles are types V and
VII. Type II affects nearly all organs, including the heart.
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Glycogen storage diseases are caused by a genetic enzyme defect that is
inherited from both parents. Normally, enzymes help convert glucose into
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glycogen for storage. Other enzymes convert the glycogen back to glucose
when quick energy is needed, as in exercise. In a person with a glycogen
storage diseases, some of these enzymes are defective, deficient, or absent.
This causes the buildup of abnormal amounts and types of glycogen in liver
and/or muscle tissues.
Since glycogen storage diseases are hereditary, the primary risk factor for is
having a family member with this disease.
Enlarged liver
Slow growth
Muscle cramps
In severe cases, infants may suffer heart failure by the age of 18 months
Enlarged liver
Anemia
Type VIII
Muscle weakness
Anemia
MRI scan – a test that uses magnetic waves to make pictures of the inside
of the body
The goal of treatment is to maintain normal blood glucose levels. This may be
done with:
In children over age two, frequent small carbohydrate feedings are given
throughout the day. This may include uncooked cornstarch. (Uncooked
cornstarch provides a steady slow-release form of glucose.)
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