Type+1+Glycogen+Storage+Disease

=Type 1 Glycogen Storage Disease =  By Tiana and Ryan

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What is GSD 1?
[|Glucose] is stored in the body in the form of [|glycogen]in our livers. It is used as a backup to __maintain a steady level of glucose__ in the body. When someone has Type 1 Glycogen Storage Disease, GSD 1, there is a missing [|enzyme]. The enzyme plays a role in releasing the glycogen to the body. If your liver is not releasing the glycogen from the [|liver], then you end up with low blood sugar. Hypoglycemia, which is caused by low blood sugar, makes a person feel tired and hungry a short while after eating. After a period of time, the liver becomes swollen and enlarged, which also makes the kidneys enlarged as well, because of the excess glycogen the liver was unable to release. The liver still functions normally, as does the kidneys, but the only difference is the release of glycogen.

Molecularly Speaking...
Our bodies use enzymes to process glycogen and glucose and to release it back into our bodies when it is needed. However, GSD 1 lacks an enzyme by the name of [|glucose-6-phosphatase]. This enzyme helps the liver to release the glycogen back into our system. The lack of activity of the enzyme causes the liver and several other organs to become enlarged but still functioning. The name truly speaks for itself. It allows glycogen in, yet doesn't release it.

Typically, GSD 1 is found within **infants** and is treated __immediately__. By testing the sugar levels of the child, performing a biopsy on the liver, and having a [|genetics test] run, the child is diagnosed early enough to treat this disease.

Because this disease is usually caught when a person is an infant, the treatments are focused on the development of a child. Treatments include feedings throughout the day of cornstarch and water mixture or soy milk. Both of these help in the release of glucose from the liver and keep the sugar levels as normal as possible. The number 1 way to keep this disease under control is to follow a __strict diet__.


 * Chronic Hunger
 * [[image:liver_damage.jpg width="275" height="281" align="right" link="http://www.nlm.nih.gov/medlineplus/ency/article/003088.htm"]]  [|Fatigue]
 * [|Enlarged Liver]
 * Enlarged Kidneys
 * Low Blood Sugar Levels

GSD 1 Isn't Your Only Problem
GSD 1 is also in a category with and may cause some of these diseases:
 * [|Hypoglycemia]
 * Kidney Enlargement
 * [|Lactic Acidemia]
 * [|Hyperlipidemia]
 * [|Hyperuricemia]

For more information about Type 1 Glycogen Storage Disease, please watch the video or visit any of the sites below: media type="youtube" key="inSkXkNK_dE&hl=en" height="355" width="425"

Pictures
http://www.brown.edu/Courses/Digital_Path/systemic_path/hepatobiliary/LV14.jpg http://www.foxriverwatch.com/liver_damage.jpg