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Ketotic hypoglycemia is substrate Limited

Presenting between the ages of 18 month and 5 years ketotic hypoglycemia is the most common form of hypoglycemia in this age group
the classical history of such patients is that a child who completely avoided an evening meal is very difficult to arouse from sleep the following morning and so again only eats poorly the next day
the child may have a fit / seizure or maybe Drowsy or comatose by noon

another mode of presentation is when parents sleep late in the night and the  childr is not able to get his breakfast early morning has the overnight fast is prolonged the patient develops the symptoms of hypoglycemia in the form of altered sensorium seizures or irritability et cetera

Alanine concentrations in children with ketotic hypoglycemia are markedly reduced and hence the infusion of alanine in the dosage of 250 milligram per kg produce a rapid rise in plasma glucose levels

Glycolytic Pathways also are intact because the infusion of glucagon induces normal glycemic response in the patients when they are in fed state

The levels of Counter regulatory hormones are also appropriately elevated
and insulin is appropriately low

The cause of ketotic hypoglycemia is usually related to deect in complex steps involved in breakdown of protein or oxidative deamination of amino acids or alanine synthesis or its eflux from muscles

Children with ketotic hypoglycemia are smaller than normal children and may have a history of transient neonatal hypoglycemia

Any decrease in muscle mass may compromise the supply of gluconeogenic substrate at a time when glucose demands are high and so decreae in muscle mass  predispose the patient to Rapid development of hypoglycemia with ketosis that represents that there has been attempted to switch to the alternative fuel supply
so it may be said that the ketotic hypoglycemia represent low end of spectrum of child's capacity to tolerate fasting

The spontaneous remission has been observed in children around the age of 8 to 9 years and it can be explained by the increase in muscle bulk which leads to increase supply of endogenous substrate alanine,
also that there is relative decrease in blood glucose requirement per unit of body mass with advancing age.

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