What organ is malfunctioning in glycogen storage disorder?

Since glycogen is primarily stored in the liver or muscle tissue, glycogen storage diseases usually affect functioning of the liver, the muscles, or both. The glycogen storage diseases that mainly affect the liver are types I, III, IV, and VI.

What is the primary side effect of glycogen storage disease?

Symptoms include a swollen belly, delayed growth, and weak muscles. Type IV or Andersen disease. People with type IV form abnormal glycogen. Experts think the abnormal glycogen triggers the body’s infection-fighting system (immune system).

Which organ is affected by von Gierke disease?

Von Gierke disease is a condition in which the body cannot break down glycogen. Glycogen is a form of sugar (glucose) that is stored in the liver and muscles. It is normally broken down into glucose to give you more energy when you need it. Von Gierke disease is also called Type I glycogen storage disease (GSD I).

Is glycogen storage disease life threatening?

Because they affect so many organ systems, GSD Type II (Pompe’s disease) and GSD Type IV (Andersen’s disease) are very hard to treat and can be fatal. Research into enzyme replacement therapy and gene therapy is promising, which may improve the outlook for the future.

What is glycogen storage disease type 1a?

Type I glycogen storage disease is inherited as an autosomal recessive genetic disorder. Glycogen storage disease type I (GSDI) is characterized by accumulation of excessive glycogen and fat in the liver and kidneys that can result in an enlarged liver and kidneys and growth retardation leading to short stature.

What is Type 1 glycogen storage disease?

How is glycogen storage disease type 1a treated?

GSDI is treated with a special diet in order to maintain normal glucose levels, prevent hypoglycemia and maximize growth and development. Frequent small servings of carbohydrates must be maintained during the day and night throughout the life. Calcium, vitamin D and iron supplements maybe recommended to avoid deficits.

How is glycogen storage disease type 1A treated?

Is glycogen storage disease a disability?

If you have been diagnosed with Glycogen Storage Disease Type II and are suffering from symptoms that make it imposable to maintain full time employment, you should file an application for Social Security Disability benefits with the Social Security Administration.