A Case of Persistent Hyperinsulinemic Hypoglycemia Treated with Diazoxide.

Kim,  Min Sun; Park,  Eun Hye; Kim,  Sun Young; Lee,  Dae Yeol

Ann Pediatr Endocrinol Metab > Volume 12(2); 2007 > Article

Case Report

Journal of Korean Society of Pediatric Endocrinology 2007;12(2): 164-167.

A Case of Persistent Hyperinsulinemic Hypoglycemia Treated with Diazoxide.

Min Sun Kim, Eun Hye Park, Sun Young Kim, Dae Yeol Lee

¹Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea. leedy@chonbuk.ac.kr
²Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea.

Abstract

Congenital hyperinsulinism (CHI), the most important cause of hyperglycemia in early infancy, is a heterogenous disease characterized by dysregulation of insulin secretion. Mutations in five proteins have been associated with CHI: sulfonyl urea receptor 1; Kir 6.2; glucokinase; glutamate dehydrogenase and mitochondrial enzyme short-chain 3-hydroxyacyl-coenzyme A dehydrogenase. Early recognition of hypoglycemia, diagnosis of CHI and appropriate management of the hypoglycemia are of the utmost importance to prevent neurologic damage. We report a case of persistent hyperinsulinemic hypoglycemia in 8-month-old male infant. This patient has no mutation in previously mentioned genes. Treatment with diazoxide was successful without any severe side effects in this patient.

Keywords: Hyperinsulinemia;Congenital;Persistent;Diazoxide