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Glycogenic hepatopathy [1] (also known as Mauriac syndrome [2]) is a rare complication of type 1 diabetes characterized by extreme liver enlargement due to glycogen deposition, along with growth failure and delayed puberty. It occurs in some children and adolescents with type 1 diabetes irrespective of their glycemic control.
A glycogen storage disease (GSD, also glycogenosis and dextrinosis) is a metabolic disorder caused by a deficiency of an enzyme or transport protein affecting glycogen synthesis, glycogen breakdown, or glucose breakdown, typically in muscles and/or liver cells.
Glycogen storage disease type I (GSD I) is an inherited disease that prevents the liver from properly breaking down stored glycogen, which is necessary to maintain adequate blood sugar levels. GSD I is divided into two main types, GSD Ia and GSD Ib, which differ in cause, presentation, and treatment.
Glycogen storage disease type IX is a hereditary deficiency of glycogen phosphorylase kinase B that affects the liver and skeletal muscle tissue. It is inherited in an X-linked or autosomal recessive manner. [1]
During exercise, cortisol helps fuel your muscles and manage physical stress by breaking down stored carbohydrates (a.k.a. glycogen) and fat, giving your body the energy it needs to perform.
Fats like adipose tissue contain about 9000 calories per kilogram. As an average adult liver weighs around 1.5 kg, and the glycogen content is probably less than half of the weight, there are maybe 2000-3000 calories worth of glycogen in your liver. The amount of glycogen in muscle and kidneys may be another kg at most.
As glycogen stores start to be depleted, the liver begins oxidizing fatty acids to ultimately yield ketone bodies, which can serve as an alternative fuel source for the brain in the absence of glucose. [3] Therefore, the combination of low glucose (hypoglycemia) and the presence of ketone bodies yields the state known as ketotic hypoglycemia.
Glycogen (black granules) in spermatozoa of a flatworm; transmission electron microscopy, scale: 0.3 μm. Glycogen is a multibranched polysaccharide of glucose that serves as a form of energy storage in animals, [2] fungi, and bacteria. [3] It is the main storage form of glucose in the human body.
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