What is the role of metabolic defects in the etiology of pediatric hypoglycemia?

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

Glucose-processing defects (Krebs cycle defects, respiratory chain defects) are rare; they interfere with the ability to appropriately generate adenosine triphosphate (ATP) from glucose oxidation. Lactate levels are high.

Defects in alternative fuel production (eg, carnitine acyl transferase deficiency, hepatic hydroxymethyl glutaryl coenzyme A [HMG CoA] lyase deficiency, long-chain and medium-chain acyl-coenzyme A dehydrogenase deficiency, variably in short-chain acyl-coenzyme A dehydrogenase deficiency) interfere with the use of fat as an energy supply, meaning that the body depends only on glucose. This becomes a problem during periods of prolonged fasting that frequently accompany gastrointestinal (GI) illness. These defects are frequently tested for during neonatal screening. Sepsis or other hypermetabolic states, such as hyperthyroidism, may cause hypoglycemia.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!