What is glycogen? What is glycogen storage disease? The carbohydrates that a person eats (such as rice and pasta) are digested and transformed into glucose that is absorbed, which raises the blood sugar in the body and provides energy to maintain various body functions and growth and development. Blood sugar in the body must be maintained in a normal range, not too high and not too low. The body will store the glucose that cannot be used for a while, and many glucose molecules gather together to become glycogen, which is mainly stored in the liver. When the body does not eat, the glycogen stored in the liver will release glucose to maintain the normal level of blood sugar in the body and ensure that no hypoglycemia occurs. The process of releasing glucose from the liver requires the participation of many enzymes. Glycogen storage disease is a deficiency of one of the enzymes in the glycogen metabolism process, resulting in impaired glycogen metabolism in the body. Depending on the missing enzyme, there are at least 10 known types of glycogen storage disorders. In the most common type I, for example, glycogen cannot release glucose due to the lack of glucose-6-phosphatase in the liver, and more and more glycogen exists in the liver, and the liver becomes larger and larger, while symptoms of hypoglycemia appear. The child will then always be hungry, have a large stomach (large liver), not grow, have easy nosebleeds, and be anemic. Long-term hypoglycemia will cause a series of changes in the body, and laboratory tests will reveal too much lactic acid in the blood and acidosis; very high blood lipids; and elevated uric acid and transaminases. If not treated, patients will develop many comorbidities. Why does a child have a genetic disorder when both parents are normal? Glycogen storage disease is a type of congenital hereditary disease that is mainly inherited in an autosomal recessive manner. This means that there is a “1” mutation in each parent’s gene that causes the disease, and they do not appear to be normal. However, when both parents pass the mutation to the child, the child carries “2” mutations in the gene and becomes sick and becomes a patient with glycogen storage disease. Is there a cure for gluconeogenic storage disease? There is no cure for glycogen storage disease. In recent years, enzyme replacement therapy has been invented for some subtypes, i.e., the enzymes are supplemented with whatever enzymes the patient lacks, but this therapy is very expensive and has not yet been implemented in China. （The treatment of glycogen storage disease (most of them) with hypoglycemic manifestations is a combination therapy based on raw corn starch. Raw corn starch-based combination therapy cannot cure the disease, but it can greatly improve the patient’s clinical symptoms and must be taken for life until a radical cure is invented. Why can raw corn starch cure glycogen storage disease? After raw corn starch is eaten into the body, it is slowly absorbed in the intestine and slowly releases glucose, which can maintain normal blood sugar for about 6 hours. With normal blood sugar, a series of pathological changes and metabolic abnormalities in the body caused by hypoglycemia, such as acidosis and elevated transaminases, will be improved. What results will be achieved after raw corn starch treatment? Most of the children with the disease have a significantly better quality of life after treatment than before. Fasting blood sugar can return to the normal range; the height increase will be faster than before, of course, some children will grow faster and some not so fast, which is related to many influencing factors: such as different subtypes, early or late treatment, whether to adhere to treatment, etc. The liver will shrink somewhat compared to before, but it is usually difficult to reach full normalcy. How to take raw corn starch? 1. Corn starch is easily available in supermarkets. Be careful to eat “corn” starch, not potato starch or any other starch. Eating old corn or ground corn is not a substitute for corn starch. 2, must eat “raw” corn starch, cooked starch or cornmeal porridge can not. 3. The dosage of raw corn starch is decided by the doctor according to the patient’s condition, so do not increase or decrease it at your own discretion. 4. One part of raw corn starch should be put into 2 parts of cool white water, stir well and drink. In winter, you should also use cool plain water, not hot water at any time to modulate. 5, eat raw corn starch must be eaten in the middle of two meals, otherwise it will not work. You can’t eat it with a piece of rice or eat starch just after eating a meal. We recommend eating starch four times a day, at 9 a.m., 3 p.m., 9 p.m. and 3 a.m. in the middle of the night. If the time of your family’s meal changes, the time of eating starch can also change, but the principle is “always eat in between meals”. The starch at 3:00 a.m. is very important and must not be omitted. After the age of 1, you can try to eat starch in small doses and more often, such as 6 times a day, but if you get diarrhea after eating starch, or even if you pull out starch, you have to reduce the dose or try again when you grow up. What should be noted in the diet of children with glycogen storage disease? 1, eat less and more meals, not too much at a time; 2, the diet is mainly rice and noodles and other food; vegetables and lean meat are not taboo and not limited; fried food is not suitable to eat too much; 3, can eat a certain amount of dairy products and fruit, but should not eat more; 4, not suitable to eat food containing sugar, should not drink sugary drinks; 5, children with high blood uric acid is not suitable to eat high purine food, such as animal offal, seafood, thick meat soup, etc.; should abstain from eating high purine food. 5. Children with high blood uric acid should not eat foods with high purine content, such as animal offal, seafood, thick meat soup, etc.; they should stop drinking alcohol; and drink more water. 6. Children with stones in the urine should drink more water, eat a low-salt diet and not eat too much meat. Why is it necessary to follow up regularly? 1, glycogen storage disease is a rare and complex disease, need experienced doctors to follow up, some cases are not easy for parents to deal with themselves 2, doctors need to regularly evaluate the effect of raw corn starch treatment, if the effect is not good, need to analyze what is the cause, by adjusting the dose or adding other treatment methods to improve the effectiveness. 3. As the patient grows older, other comorbidities of glycogen storage disease will become apparent, such as adenomas on the liver, blood in the urine, positive urine protein, osteoporosis, gout, etc., which need to be detected and treated accordingly. Can patients with glycogen storage disease marry and have children when they grow up? Will their children also have the same disease? With proper treatment and no serious complications, patients with glycogen storage disease can get married and have a normal sex life. If the marriage partner is not a close relative, the vast majority of children will not have the same disease, but will simply be carriers of the gene for gluconeogenic storage disease.