Glucose is the principal substrate for energy metabolism and disorders that affect its availability or use can result in hypoglycaemia. A normal blood glucose level is maintained by the interplay of glucose production and glucose utilisation. The key sources of glucose production include glucose ingestion from the diet and the adaptive metabolic processes of glycogenolysis and gluconeogenesis. Hypoglycaemia is common in clinical practice and can be a manifestation of many underlying conditions. It is a biochemical finding and not a diagnosis. At a genetic level, hypoglycaemia can be due to many different genetic disorders both metabolic and endocrine. Some of these genetic disorders present with severe and profound hypoglycaemia in the newborn period yet others can be mild and subtle. Endogenous glucose supply depends on the breakdown of glycogen stores for the first a few hours after birth, followed by synthesis of glucose from lactate, glycerol and amino acids. When glucose levels begin to decline during fasting states, hormonal and metabolic pathways are triggered to raise the blood glucose level. Hormonal equilibrium is maintained by the appropriate response of insulin and counterregulatory hormones such as epinephrine, glucagon, growth hormone and cortisol. The central nervous system integrates the counterregulatory hormonal responses, and coordinates the neuroendocrine, autonomic and behavioural responses to hypoglycaemia.
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Last date updated on July, 2014