A Case of Persistent Hyperinsulinemic Hypoglycemia Treated with Diazoxide.
- Author:
Min Sun KIM
1
;
Eun Hye PARK
;
Sun Young KIM
;
Dae Yeol LEE
Author Information
1. Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea. leedy@chonbuk.ac.kr
- Publication Type:Case Report
- Keywords:
Hyperinsulinemia;
Congenital;
Persistent;
Diazoxide
- MeSH:
Congenital Hyperinsulinism*;
Diagnosis;
Diazoxide*;
Glucokinase;
Glutamate Dehydrogenase;
Humans;
Hyperglycemia;
Hyperinsulinism;
Hypoglycemia;
Infant;
Insulin;
Male;
Oxidoreductases;
Urea
- From:Journal of Korean Society of Pediatric Endocrinology
2007;12(2):164-167
- CountryRepublic of Korea
- Language:Korean
-
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.
