Glycogen Storage Disease type I (GSD I), also known as von Gierke disease, is a genetic disorder that affects the body's ability to store and use glycogen, a form of sugar. This condition is caused by a deficiency in the enzyme glucose-6-phosphatase, which is essential for converting glycogen into glucose. As a result, individuals with GSD I experience low blood sugar levels, especially during fasting. Symptoms of GSD I can include enlarged liver, growth delays, and increased risk of infections. Management typically involves maintaining normal blood sugar levels through frequent meals and a diet rich in carbohydrates. Regular monitoring and medical care are crucial for those affected by this condition.