Soonchunhyang Med Sci.  2014 Jun;20(1):56-59.

A Case of 2-Month-Old Infant with Persistent Hyperinsulinemic Hypoglycemia Presenting as Atonic Seizure

• Kim JW
• Kim DH
• Kim SS

Abstract

Congenital hyperinsulinism is the most frequent cause of severe, persistent hypoglycemia in infancy and childhood. It is caused by an inappropriate insulin secretion from the pancreatic beta-cells secondary to various genetic disorders. Recognition of this entity becomes important due to the fact that hypoglycemia is very severe and frequent and that it may lead to severe neurological damage in the infant manifesting as mental or psychomotor retardation or even a life-threatening events if not recognized and treated effectively in time. Hypoglycemias can be detected by seizures, fainting, or any other neurological symptoms in the neonatal period or later, usually within the first two years of life. Hypoglycemias must be rapidly and intensively treated to prevent severe and irreversible brain damages. Next, a treatment to prevent the recurrence of hypoglycemia must be set, which may include frequent and glucose-enriched feeding, diazoxide and octreotide. We report a case of congenital hyperinsulinemia in a 2 months old infant presenting as atonic seizure which has been treated with diazoxide.

Keyword

Congenital hyperinsulinism; Hypoglycemia; Seizures

MeSH Terms

Brain
Congenital Hyperinsulinism*
Diazoxide
Humans
Hyperinsulinism
Hypoglycemia
Infant*
Insulin
Octreotide
Recurrence
Seizures*
Syncope
Diazoxide
Insulin
Octreotide