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文献名称

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PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA WITH LEFT VENTRICULAR HYPERTROPHY AND DYSRHYTHMIA: A Case Report
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文献简介

出版社:Fetal and Pediatric Pathology

作者:Ali Bulbul, Fatih Bolat, and Serdar Comert

编号:10.3109/15513811003777334

关键字:newborn, persistent hyperinsulinemic hypoglycemia, left ventricular hyper- trophy, dysrhythmia

年份:2010点击量:264

文献摘要

          Persistent hyperinsulinemic hypoglycemia (PHH) is the most common cause of persistent hypoglycemia during the neonatal period and infancy, characterized by insulin hypersecretion. Persistent hyperinsulinemic hypoglycemia was first described by McQuarre in 1954 . Persistent hyperin-sulinemic hypoglycemia, also called “nesidioblastosis,” presents clinically in first 3 months of age. Incidence is reported to be 1/50,000 births . Increased prevalence among populations with increased rates of consanguinity suggests that the disease is genetic rather than sporadic. Prevalence in some populations is found to be 1/2,700. Hyperinsulinism is due to genetic defects in the regulation of insulin secretion.

         Hyperinsulinemic states can be rarely associated with a hypertrophic cardiomyopathy . This is possibly related to the growth factor-like properties of insulin . However, PPH was reported only in two newborns
concomitant with ventricular hypertrophy .
          Severe hypoglycemia, secondary to hyperinsulinism, may lead to intractable convulsions and brain damage. The mainstay of treatment is to achieve normoglycemia and to prevent brain damage . In cases unresponsive to the medical treatment, a subtotal or total pancreatectomy is performed . A newborn with left ventricular hypertrophy and persistent hypoglycemia unresponsive to medical treatment, who underwent pancreatectomy and was diagnosed to have PHH, is presented.

 

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