Congenital Hyperinsulinism

  • N. Scott Adzick


Congenital hyperinsulinism (HI) is a rare derangement of glucose metabolism that occurs with an estimated incidence of 1–1.4 in 50,000 live births, accounting for about 80–120 new cases in the United States each year. Higher rates of 1 in 2,500 live births have been reported in areas of high consanguinity, such as the Arabian Peninsula. Inappropriate over secretion of insulin is the hallmark of HI. The old term “nesidioblastosis” should be discarded. HI is the most common cause of persistent hypoglycemia in neonates and can lead to seizures and irreversible brain damage. Pancreatectomy for management of persistent infantile hypoglycemia was first performed at The Children’s Hospital of Philadelphia (CHOP) in 1950.


Common Bile Duct Pancreatic Head KATP Channel Focal Lesion Normal Pancreas 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Suggested Reading

  1. Adzick NS, Thornton PS, Stanley CA, et al. A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy. J Pediatr Surg. 2004;39:270–5.PubMedCrossRefGoogle Scholar
  2. De Lonlay P, Poggi-Travert F, Founet JC, et al. Clinical features of 52 neonates with hyperinsulinism. N Engl J Med. 1999;340: 1169–75.CrossRefGoogle Scholar
  3. DeLeon DD, Stanley CA. Mechanisms of disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Nat Clin Prac. 2007;3:57–68.CrossRefGoogle Scholar
  4. Hardy OT, Hernandez-Pampaloni M, Saffer JR, et al. Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism. J Clin Endocrinol Metab. 2007;92:4706–11.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of SurgeryChildren’s Hospital of Philadelphia, University of Pennsylvania School of MedicinePhiladelphiaUSA

Personalised recommendations