Glycogen Storage Disease Type 1
GSD is a genetic disorder in which abnormal amounts of glycogen are stored in the body. Glycogen is the storage form of glucose coming from the bloodstream, which is later used and converted into energy through glycolysis. There are many types of glycogen storage disease however, Type I is the most common, as 90% of the cases are associated with this type. In contrast to diabetes, GSD Tyoe I results in low blood sugar as glycogen cannot be converted back into glucose, as well as impaired growth, enlarged liver and kidneys, and high levels or lactate and fats in the blood. Type I is referred to as von Gierke's disease, and inflicts about 200,000 people in the US. The disease is a result of the lack of an enzyme necessary to break down glycogen into glucose. This is obviously problematic, as glucose is an essential molecule to human life. One of few treatments is frequent feedings of carbohydrates to maintain the even blood sugar, as the enzyme is only activated after not eating for upwards of 3 hours. Von Gierke's disease is scary, and epitomizes the incredible importance glucose plays in the body.