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If the blood glucose level falls to dangerously low levels (as during very heavy exercise or lack of food for extended periods), the alpha cells of the pancreas release glucagon, a peptide hormone which travels through the blood to the liver, where it binds to glucagon receptors on the surface of liver cells and stimulates them to break down glycogen stored inside the cells into glucose (this ...
In glycogen storage diseases, however, glycogen cannot be properly broken down, leading to inappropriately decreased glucose levels in a fasting state, and thus hypoglycemia. [3] The glycogen storage diseases associated with hypoglycemia include type 0 , type I , type III , and type IV , as well as Fanconi syndrome .
Mechanisms that restore satisfactory blood glucose levels after extreme hypoglycemia (below 2.2 mmol/L or 40 mg/dL) must be quick and effective to prevent extremely serious consequences of insufficient glucose: confusion or unsteadiness and, in the extreme (below 0.8 mmol/L or 15 mg/dL) loss of consciousness and seizures.
When the blood glucose level is too high, glucose can be stored in the liver as glycogen. When the level is too low, the glycogen can be catabolised and glucose may re-enter the blood stream. The catabolic process occurs at the nonreducing end of glycogen.
In patients with type 1 diabetes mellitus, as plasma glucose levels fall, insulin levels do not decrease – they are simply a passive reflection of the absorption of exogenous insulin. Also, glucagon levels do not increase. Therefore, the first and second defenses against hypoglycemia are already lost in established type 1 diabetes mellitus. [2]
Studies show that glycogen also plays a role in muscle repair and growth. “When combined with protein, carbohydrates can enhance muscle protein synthesis,” says Samantha DeVito, M.S., RD, CDN ...
If the child is underweight, a daily nutritional supplement may be recommended. For children with ketotic hypoglycemia due to a glycogen storage disease, overnight cornstarch can help maintain blood glucose levels and reduce the incidence of hypoglycemic episodes. [2] [9]
The purpose of the off-label 'mini-dose' is to avoid an emergency condition that may require glucagon rescue. This might be needed in cases such as when a diabetic child is injected with insulin before breakfast, eats, and then vomits and cannot eat again: with the injected insulin working its way into the bloodstream and no carbohydrate to balance, there may soon be a hypoglycemic emergency.
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