Congenital hyperinsulinism - A review of the disorder and a discussion of the anesthesia management

Olga T. Hardy, Ronald S. Litman

Research output: Contribution to journalReview article

8 Scopus citations

Abstract

Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infants and children. In most affected infants, CHI is caused by a specific genetic defect that results in the altered expression of pancreatic beta cells causing unregulated oversecretion of insulin. Infants with CHI may have either focal or diffuse abnormalities of the pancreatic β-cells. Both forms of CHI manifest as hypoglycemia, usually in the early newborn period. Focal disease can be treated effectively with surgical resection of the affected area, resulting in a total cure or rendering the patient amenable to medical management. Most children with diffuse disease are unresponsive to medical therapy, and require near-total pancreatectomy. At The Children's Hospital of Philadelphia, we have developed a multidisciplinary program for diagnosis and treatment of CHI. Anesthesiologists have played an integral role in the perioperative care of these infants, which includes diagnostic procedures, partial or near-total pancreatectomy, and postoperative pain management. In this review, we describe the clinical features, diagnostic methods and anesthetic concerns in children with CHI.

Original languageEnglish (US)
Pages (from-to)616-621
Number of pages6
JournalPaediatric anaesthesia
Volume17
Issue number7
DOIs
StatePublished - Jul 1 2007

Keywords

  • Congenital hyperinsulinism
  • Hypoglycemia of infancy
  • Nesidioblastosis
  • Pancreatic islets
  • Persistent hyperinsulinemia
  • Positron emission tomography
  • [18F]-DOPA

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Anesthesiology and Pain Medicine

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