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


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