Starvation their normal body weight. Once the loss reaches 40% death is almost inevitable. Under normal metabolic conditions, the human body relies on free blood glucose as its primary energy source. The level of blood sugar is tightly regulated; as blood glucose is consumed, the pancreas releases glucagon, a hormone that stimulates the liver to convert stored glycogen into glucose. The glycogen stores are ordinarily replenished after every meal, but if the store is depleted before it can be replenished, the body enters hypoglycemia, and begins the starvation response. After the exhaustion of the glycogen reserve, and for the next 2–3
Glycogen storage disease type 0 of asymptomatic and oligosymptomatic siblings in several glycogen-storage disease type 0 families has suggested that glycogen-storage disease type 0 is underdiagnosed. The major morbidity is a risk of fasting hypoglycemia, which can vary in severity and frequency. Major long-term concerns include growth delay, osteopenia, and neurologic damage resulting in developmental delay, intellectual deficits, and personality changes. No sexual predilection is observed because the deficiency of glycogen synthetase activity is inherited as an autosomal recessive trait. Glycogen-storage disease type 0 is most commonly diagnosed during infancy and early childhood. Glycogen storage disease type 0 Glycogen storage disease type 0 is a