J Korean Soc Pediatr Endocrinol.  1998 Nov;3(2):231-236.

Nesidioblastosis in Neonate with Persistent Hyperinsulinemic Hypoglycemia

• Whang IT
• Kim HS
• Han HS

Abstract

Nesidioblastosis, also known as persistent hyperinsulinemic hypoglycemia of infancy(PHHI) or familial hyperinsulinsm, is the most common cause of recurrent severe hypoglycemia in infancy. It is an autosomal recessive disorder characterized by irregular insulin secretion leading to inappropriately raised plasma insulin concentration compared to blood glucose levels. Recently, mutations in the sulfonylurea receptor(SUR) have been described in association with PHHI. The mainstay of medical treatment is glucose infusion and diazoxide or long acting somatostatin. If medical treatment fails in preventing hypoglycemia, near total pancreatectomy is recommended. We report one case of nesidioblastosis cured by near total pancreatectomy with brief review of literatures.

Keyword

Persistent hyperinsulinemic hypoglycemia of infancy; Nesidioblastosis

MeSH Terms

Blood Glucose
Congenital Hyperinsulinism*
Diazoxide
Glucose
Humans
Hypoglycemia
Infant, Newborn*
Insulin
Nesidioblastosis*
Pancreatectomy
Plasma
Somatostatin
Blood Glucose
Diazoxide
Glucose
Insulin
Somatostatin