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KMID : 0648420070120020164
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Journal of Korean Society of Pediatric Endocrinology
2007 Volume.12 No. 2 p.164 ~ p.167
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A Case of Persistent Hyperinsulinemic Hypoglycemia Treated with Diazoxide
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Kim Min-Sun
Lee Dae-Yeol
Park Eun-Hye
Kim Sun-Young
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Abstract
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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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KEYWORD
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Hyperinsulinemia, Congenital, Persistent, Diazoxide
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