Persistent hyperinsulinemic hypoglycemia in infants

Abstract

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of recurrent hypoglycemia in neonates and infants. It is a disorder of glucose homeostasis characterized by unregulated hyperinsulinemia and profound hypoglycemia. PHHI may be differentiated from other causes of hypoglycemia by demonstrating the persistence of inappropriately high insulin levels regardless of blood glucose concentration. Prompt recognition and treatment of PHHI is critical because uncorrected hypoglycemia in the newborn period is associated with permanent damage to the central nervous system and subsequent mental retardation. The aim of therapy is to maintain euglycemia to protect the developing brain from possible damage. Despite recent advances in medical treatment, subtotal pancreatectomy is often necessary. This article will review the function of insulin, glucagon, and somatostatin, pathophysiology of hyperinsulinism, clinical manifestations, differential diagnosis, management, and reported neurologic outcomes of newborns and infants with PHHI. Ce—feature articleLink to www.nainr.com, click “CE Articles” to access the approved and accredited Continuing Education Questions for this featured article. Approval and accreditation are through Mosby Continuing Education and Training. Specific instructions and information on the number of credits available and the testing processes can be viewed on the websiteCE ArticlesCE Articles.