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glycogen synthase deficiency or glycogen storage disorders type 0

Glycogen storage disorder type 0, there is inability to synthesise glycogen
Hence liver glycogen reserves are very diminished

There is hypoglycemia and hyper ketonemia

After having a field there occurs hyperglycemia with glucose urea because of the inability Tu store glucose load in the form of glycogen

In fasting States there is hypoglycemia the levels of Counter regulatory hormones including catecholamines are very appropriately elevated and insulin levels Get low

Liver is normal sized

Protein rich feedings at frequent intervals result in clinical improvement such that growth velocity also improves

This condition closely mimics ketotic hypoglycemia and should be considered in its differential diagnosis

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