What is the role of glucose production in the pathogenesis of pediatric hypoglycemia?

Updated: Feb 19, 2018
  • Author: Robert P Hoffman, MD; Chief Editor: Sasigarn A Bowden, MD  more...
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Answer

Inadequate glucose stores are associated with prematurity, infants who are small for gestational age, malnutrition, and ketotic hypoglycemia. After insulin treatment in diabetes, these disorders are the most common causes of hypoglycemia. The first three of these should be readily apparent based on the clinical situation. Ketotic hypoglycemia, which usually affects small, thin children aged 18 months to 6 years, is usually caused by disrupted food intake. Ketotic hypoglycemia is a diagnosis of exclusion, made after other causes of hypoglycemia are ruled out.

Glycogen synthase deficiency (glycogen-storage disease type 0) is associated with fasting hypoglycemia because of the liver’s inability to store glucose in the immediate postprandial state. Thus, the glucose load from the meal is anaerobically metabolized rather than stored for later use. In this disorder, plasma glucose and lactate levels are high in the immediate postprandial state. Glycogen synthase deficiency must be considered before the diagnosis of ketotic hypoglycemia is assigned.

Disorders of hepatic glucose production include glucose-6-phosphatase deficiency (glycogen-storage disease type Ia); glucose 6-phosphate translocase deficiency (glycogen storage disease type 1b); debrancher deficiency (glycogen-storage disease type III); hepatic phosphorylase deficiency (glycogen-storage disease type VI); glycogen synthase deficiency; fructose 1,6 diphosphatase deficiency; phosphoenolpyruvate deficiency; pyruvate carboxylase deficiency; galactosemia; hereditary fructose intolerance; and maple syrup urine disease. These disorders interfere in glucose production through various defects, including blockage of glucose release or synthesis or blockage or inhibition of gluconeogenesis. Children with these diseases may adapt to their hypoglycemia because of its chronicity.

Hormonal abnormalities include panhypopituitarism, growth hormone deficiency, and cortisol deficiency (primary or secondary). As described above, growth hormone and cortisol play important roles in generating alternative fuels and stimulating glucose production. Because they are easily treatable abnormalities, early recognition is important.


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