Type I Glycogen Storage Disease, also known as Von Gierke disease, is a genetic disorder that affects the body's ability to break down glycogen into glucose. This condition is caused by a deficiency in the enzyme glucose-6-phosphatase, which is essential for converting glycogen stored in the liver into usable glucose. As a result, individuals with this disease experience low blood sugar levels, especially during fasting. Symptoms of Type I Glycogen Storage Disease can include severe hypoglycemia, growth delays, and an enlarged liver, known as hepatomegaly. Management typically involves dietary changes, such as frequent meals and the use of cornstarch to maintain stable blood sugar levels. Regular monitoring and medical care are crucial for managing this condition effectively.