How is glycogen storage disease treated?
Treatment consists of taking regular doses of uncooked cornstarch and/or nutrition supplements. Cornstarch is a complex carbohydrate that is difficult for the body to digest; therefore it maintains normal blood sugar levels for a longer period of time than most carbohydrates in food.
How is von Gierke’s disease treated?
The goal of treatment is to avoid low blood sugar. Eat frequently during the day, especially foods that contain carbohydrates (starches). Older children and adults may take cornstarch by mouth to increase their carbohydrate intake.
Is glycogen storage disease curable?
How is glycogen storage disease treated? There is currently no cure for GSD. After diagnosis, children with GSD are usually cared for by several specialists, including specialists in endocrinology and metabolism.
What is the life expectancy of someone with glycogen storage disease?
Q: What is the life expectancy of a person with glycogen storage disease? A: The life expectancy of persons with glucose-6-phosphatase deficiency, debrancher deficiency, and with liver phosphorylase deficiency is probably somewhat reduced although many do quite well.
What is the most common type of glycogen storage disease?
Type I (Von Gierke disease) – this is the most common type of glycogen storage disease, and accounts for 90% of all glycogen storage disease cases. Type II (Pompe’s disease, acid maltase deficiency)
What are the symptoms of glycogen storage disease?
General symptoms of GSD may include:
- Not growing fast enough.
- Not feeling comfortable in hot weather (heat intolerance)
- Bruising too easily.
- Low blood sugar (hypoglycemia)
- An enlarged liver.
- A swollen belly.
- Weak muscles (low muscle tone)
- Muscle pain and cramping during exercise.
What is the most common glycogen storage disease?
How do you deplete glycogen stores quickly?
Exercise helps a person deplete the glycogen stores in their body. In most cases, the glycogen stores become replenished when a person eats carbs. If a person is on a low-carb diet, they will not be replenishing their glycogen stores. It can take some time for the body to learn to use fat stores instead of glycogen.
How is glycogen storage disease type 1 treated?
The treatment of type I glycogen storage disease is focused on correcting the metabolic changes in the body and promoting the growth and development of the child. A combination of uncooked cornstarch mixed in water, soy formula, or soy milk is often recommended.
What is the most serious type of glycogen storage disease?
How common is von Gierke disease?
Type I glycogen storage disease (GSD I), also known as von Gierke’s disease, is the most common form of glycogen storage disease, accounting for 25% of all cases. It is an inherited disorder that affects the metabolism – the way the body breaks food down into energy.
What is glycogen storage disease Type IX?
Glycogen storage disease type IX (also known as GSD IX) is a condition caused by the inability to break down a complex sugar called glycogen. The different forms of the condition can affect glycogen breakdown in liver cells or muscle cells or sometimes both. A lack of glycogen breakdown interferes with the normal function of the affected tissue.
How is glycogen storage disease ( GSD ) treated?
For types of GSD that involve the liver, treatment is aimed at keeping the right level of glucose in the blood. This is often enough to maintain the cells fuel needs and prevent long-term complications associated with poorly controlled GSD. Treatment consists of taking regular doses of uncooked cornstarch and/or nutrition supplements.
Can a child be diagnosed with glycogen storage disease?
However, GSD types VI and IX can have very mild symptoms and may be underdiagnosed. Most of the severe forms of GSD are diagnosed in babies and children. Some of the milder types might not be found until the person is an adult. Cleveland Clinic is a non-profit academic medical center.
How does glycogen storage disease affect the body?
Glycogen storage disease (GSD) is a rare condition that changes the way the body uses and stores glycogen, a form of sugar or glucose. Glycogen is a main source of energy for the body.