Nesidioblastosis 1례

초록

Nesidioblastosis is rare but the most common cause of persistent hyperinsulinemic hypoglycemia in infancy and childhood. This disease is usually sporadic, occurs also in familial clusters and an autosomal recessive transmission has been proposed. In this condition, the endocrine abnormality is inappropriate insulin secretion, which leads to hyperinsulinemic hypoglycemia. Since the hypoglycemia may be exceedingly difficult to control and associated with a high incidence of brain damage and subsequent mental retardation, it is a diagnostically important disease. The current management remains surgery—subtotal or near total pancreatectomy. We experienced a case of nesidioblastosis in a male newborn infant. In his fourth postnatal day, this patient presented with seizure, and clinical diagnosis was established by demonstrating inappropriate elevation of serum insulin (>10 µU/mL) in the presence of hypoglycemia. Initially, hypoglycemia was controlled with intravenous infusion of glucose and hydrocortisone but it recurred. Therefore, this patient underwent near total pancreatectomy and became normoglycemic.

키워드