Hyperinsulinism in the Neonate

Clin Perinatol. 2018 Mar;45(1):61-74. doi: 10.1016/j.clp.2017.10.007. Epub 2017 Dec 6.

Abstract

Hyperinsulinism (HI) is the leading cause of persistent hypoglycemia in infants. Prompt recognition and treatment, independent of whether infants have transient or permanent HI, are essential to decrease risk of neurologic damage. The most common form of congenital HI is due to inactivating mutations of the β-cell ATP-sensitive potassium (KATP) channel (KATP-HI) and is typically diazoxide unresponsive. KATP-HI occurs in diffuse and focal forms. Distinguishing between the 2 forms is crucial, because pancreatectomy is curative in the focal form but palliative in the diffuse form. The 18-fluoro-L-3,4-dihydroxyphenylalanine PET scan has revolutionized HI management by allowing accurate localization of focal lesions prior to surgery.

Keywords: Hyperinsulinism; Hypoglycemia; Insulin; Neonate; Pancreas; Pancreatectomy.

Publication types

  • Review

MeSH terms

  • Abnormalities, Multiple
  • Antihypertensive Agents / therapeutic use
  • Beckwith-Wiedemann Syndrome / complications
  • Congenital Hyperinsulinism / diagnosis*
  • Congenital Hyperinsulinism / genetics
  • Congenital Hyperinsulinism / therapy
  • Diazoxide / therapeutic use
  • Face / abnormalities
  • Genetic Testing
  • Hematologic Diseases / complications
  • Humans
  • Hyperinsulinism / diagnosis*
  • Hyperinsulinism / etiology
  • Hyperinsulinism / therapy
  • Infant, Newborn
  • Sotos Syndrome / complications
  • Stress, Physiological
  • Turner Syndrome / complications
  • Vestibular Diseases / complications

Substances

  • Antihypertensive Agents
  • Diazoxide

Supplementary concepts

  • Kabuki syndrome