Hyperinsulinemic Hypoglycemia

Pediatr Clin North Am. 2015 Aug;62(4):1017-36. doi: 10.1016/j.pcl.2015.04.010. Epub 2015 May 13.

Abstract

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.

Keywords: 18F-l-dihydroxyphenylalanine positron emission tomographic scan; Diazoxide; Hyperinsulinism; Hypoglycemia; Mammalian target of rapamycin (mTOR) inhibitor; Octreotide; Pancreatectomy.

Publication types

  • Review

MeSH terms

  • Blood Glucose / analysis
  • Brain Diseases / etiology
  • Brain Diseases / prevention & control
  • Congenital Hyperinsulinism / complications
  • Congenital Hyperinsulinism / diagnosis*
  • Congenital Hyperinsulinism / genetics
  • Congenital Hyperinsulinism / therapy*
  • Diagnostic Imaging
  • Genetic Testing
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Infant, Newborn
  • Insulin / metabolism*
  • Insulin Secretion
  • Insulin-Secreting Cells / metabolism*
  • Pancreatectomy
  • Receptors, Glucagon / antagonists & inhibitors
  • Sirolimus / therapeutic use

Substances

  • Blood Glucose
  • Immunosuppressive Agents
  • Insulin
  • Receptors, Glucagon
  • Sirolimus