J Korean Soc Pediatr Endocrinol.
2007 Dec;12(2):164-167.
A Case of Persistent Hyperinsulinemic Hypoglycemia Treated with Diazoxide
- Affiliations
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- 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.
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