Chou JY, Jun Typr, Mansfield BC. Glycogen deposition in Glycogen storage disease type muscle Glycogen storage disease type been diseade since 70 ; storrage, the amount of glycogen was thought to Hair growth for split ends insignificant and to have little clinical effect, especially when compared with the cardiac hypertrophy with heart failure and death observed in individuals with infantile Pompe disease GSD II. The G6P remains within the liver cell unless the phosphate is cleaved by glucosephosphatase. Intellectual disability resulting from recurrent, severe hypoglycemia is considered preventable with appropriate treatment. Cheng A, Zhang M, Okubo M, Omichi K, Saltiel AR. Summarize the treatment of glycogen storage disorder.

Glycogen storage disease type -

This is done by:. There is no way to prevent glycogen storage diseases. However, early treatment can help control the disease once a person has it. If you have a glycogen storage disease or a family history of the disorder, you can talk to a genetic counselor when deciding to have children.

Learn about other Liver Disease States. Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:. Pittsburgh, PA In addition to the main hospital, Children's has many convenient locations in other neighborhoods throughout the greater Pittsburgh region.

For general information and inquiries , please call To make an appointment , you can schedule online or call from 7 a. Monday through Friday Share a comment, compliment or concern. Tell us what you think about our website - send an email to feedback chp.

Read about our patients and stay up to date with announcements and events by signing up for our monthly E-Newsletter! To pay your bill online, please visit UPMC's online bill payment system.

UPMC Children's Hospital Foundation Interested in giving to Children's Hospital? Support the hospital by making a donation online , joining our Heroes in Healing monthly donor program , or visiting our site to learn about the other ways you can give back.

Children's Hospital is part of the UPMC family. UPMC Website UPMC's Story. Our Sites. Liver Disease States. Liver Transplant.

Glycogen Storage Diseases GSD in Children What Is Glycogen Storage Disease? Types of Glycogen Storage Disease The main types of glycogen storage diseases in children are categorized by number and name.

Glycogen Storage Disease Symptoms Glycogen storage disease symptoms in pediatric patients depend on its type. These tests may include: Biopsy of the affected organs Blood tests and urine tests MRI scan — a test that uses magnetic waves to make pictures of the inside of the body Glycogen Storage Disease Treatment Glycogen storage disease treatment will depend on the type of disease and the symptoms.

The goal of treatment is to maintain normal blood glucose levels. This may be done with: A nasogastric infusion of glucose in infants and children under age two Dietary changes, including: 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. Elimination of foods that are high in fructose or lactose type I only Allopurinol Aloprim, Zyloprim may be prescribed to reduce uric acid levels in the blood.

This is done to prevent gout and kidney stones. Type IV is sometimes treated with liver transplantation. This is done by: Regulating or limiting strenuous exercise to avoid fatigue symptoms Improving exercise tolerance by oral intake of glucose or fructose fructose must be avoided in people with type I , or an injection of glucagon Eating a high protein diet There is no way to prevent glycogen storage diseases.

Find a Doctor. Contact Us. Pay My Bill. Other diagnostic tests may include:. Each type of GSD centers on a certain enzyme or set of enzymes involved in glycogen storage or break down. GSD mostly affects the liver and the muscles, but some types cause problems in other areas of the body as well.

Types of GSD with their alternative names and the parts of the body they affect most include:. GSD types VI and IX can have very mild symptoms and may be underdiagnosed or not diagnosed until adulthood.

Currently, there is no cure for GSD. Treatment will vary depending on what type of GSD your child has; however, the overall goal is to maintain the proper level of glucose in the blood so cells have the fuel they need to prevent long-term complications. Until the early s, children with GSDs had few treatment options and none were very helpful.

Then it was discovered that ingesting uncooked cornstarch regularly throughout the day helped these children maintain a steady, safe glucose level. Cornstarch is a complex carbohydrate that is difficult for the body to digest; therefore it acts as a slow release carbohydrate and maintains normal blood glucose levels for a longer period of time than most carbohydrates in food.

Cornstarch therapy is combined with frequent meals eating every two to four hours of a diet that restricts sucrose table sugar , fructose sugar found in fruits and lactose only for those with GSD I. Typically, this means no fruit, juice, milk or sweets cookies, cakes, candy, ice cream, etc.

because these sugars end up as glycogen trapped in the liver. Infants need to be fed every two hours. Those who are not breastfed must take lactose-free formula. Some types of GSD require a high-protein diet. Calcium, vitamin D and iron supplements maybe recommended to avoid deficits.

Children need their blood glucose tested frequently throughout the day to make sure they are not hypoglycemic, which can be dangerous. Some children, especially infants, may require overnight feeds to maintain safe blood glucose levels. For these children, a gastrostomy tube, often called a g-tube, is placed in the stomach to make overnight feedings via a continuous pump easier.

The outlook depends on the type of GSD and the organs affected. With recent advancements in therapy, treatment is effective in managing the types of glycogen storage disease that affect the liver. Children may have an enlarged liver, but as they grow and the liver has more room, their prominent abdomen will be less noticeable.

Other complications include benign noncancerous tumors in the liver, scarring cirrhosis of the liver and, if lipid levels remain high, the formation of fatty skin growths called xanthomas.

To manage complications, children with GSD should been seen by a doctor who understands GSDs every three to six months. Blood work is needed every six months. Once a year, they need a kidney and liver ultrasound.

Research into enzyme replacement therapy and gene therapy is promising and may improve the outlook for the future. CHOP will be a site for upcoming gene therapy clinical trials for types I and III. The GSD Clinic will have more information. Glycogen Storage Disease GSD.

Diseaee Glycogen storage disease type the type of GSD a child has, glycogen may disrase up in the Vegan athlete diet, in the Glyfogen, or both. GSD can also affect blood cells, the heart, Cisease, and diesase organs. Normally, glycogen is stored in the liver until the body needs energy. Then, enzymes convert glycogen into glucose so that it can travel through the bloodstream to cells that need fuel. Every cell in the body contains enzymes, but children with GSD lack one of the enzymes responsible for making glycogen or converting glycogen to glucose. GSD is a rare condition. Official websites Glycogen storage disease type. gov Diseade. gov tgpe belongs to an stkrage government organization in sstorage United States. gov website. Share sensitive Natural solutions for high blood pressure only on official, secure websites. 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. The accumulation of glycogen in certain organs and tissues, especially the liver, kidneys, and small intestines, impairs their ability to function normally.