Nesidioblastosis in Neonate with Persistent Hyperinsulinemic Hypoglycemia.
- Author:
Il Tae WHANG
;
Ho Seong KIM
;
Ho Seong HAN
- Publication Type:Original Article
- Keywords:
Persistent hyperinsulinemic hypoglycemia of infancy;
Nesidioblastosis
- MeSH:
Blood Glucose;
Congenital Hyperinsulinism*;
Diazoxide;
Glucose;
Humans;
Hypoglycemia;
Infant, Newborn*;
Insulin;
Nesidioblastosis*;
Pancreatectomy;
Plasma;
Somatostatin
- From:Journal of Korean Society of Pediatric Endocrinology
1998;3(2):231-236
- CountryRepublic of Korea
- Language:Korean
-
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.
