In hyperinsulinemic hypoglycemia (HH) there is dysregulation of insulin secretion from pancreatic β-cells. Insulin secretion becomes inappropriate for the level of blood glucose leading to severe hypoglycemia. HH is associated with a high risk of brain injury because insulin inhibits lipolysis and ketogenesis thus preventing the generation of alternative brain substrates (such as ketone bodies). Hence HH must be diagnosed as soon as possible and the management instituted appropriately to prevent brain damage. This article reviews the mechanisms of glucose physiology in the newborn, the mechanisms of insulin secretion, the etiologic types of HH, and its management.
Key points
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Hyperinsulinemic hypoglycemia (HH) is characterized by the inappropriate secretion of insulin from pancreatic β-cells and is a major cause of hypoglycemic brain injury.
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It is recommended that blood glucose concentrations be maintained greater than 3.5 mmol/L in patients with HH because of the lack of alternative substrates for the brain to use.
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Genetic testing for mutations in the genes ABCC8/KCNJ11 for congenital HH helps in determining if the child has focal or diffuse disease.
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The 18F- l -3,4-dihydroxyphenylalanine ( 18 F-DOPA)-PET/computed tomographic (CT) scan is now the gold standard for the accurate preoperative localization of the focal lesion.
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Novel therapeutic drugs such as mammalian target of rapamycin (mTOR) inhibitors (like sirolimus) and glucagon-like peptide-1 (GLP-1) receptor antagonist offer new medical treatment options for severe diffuse disease, decreasing the requirement for a near-total pancreatectomy.
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