Girls often have ovarian failure during puberty and adulthood, and only a few are able to conceive naturally. In glycogen storage diseases, abnormal types or amounts of glycogen are deposited in the body tissues, mostly the liver. Symptoms result from the accumulation of glycogen or its by-products or from an inability to produce glucose when needed. The age at which symptoms start and their severity vary considerably among these diseases because different enzymes are affected in each disease. The diagnosis is made when an examination of a sample of tissue, usually muscle or liver, determines that a specific enzyme is missing.
Treatment depends on the type of glycogen storage disease.
For many people, eating several small carbohydrate-rich meals every day helps prevent blood sugar levels from dropping. For some young children, giving uncooked cornstarch every 4 to 6 hours around the clock can also relieve the problem. Sometimes carbohydrate solutions are given through a stomach tube all night. Drug treatment to prevent this accumulation is often necessary. In some types of glycogen storage disease, the amount of exercise children do must be limited to reduce muscle cramps.