Hyperinsulinemic Hypoglycemia – The Molecular Mechanisms

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Hyperinsulinemic Hypoglycemia – The Molecular Mechanisms

Under normal physiological conditions, pancreatic β-cells secrete insulin to maintain fasting blood glucose levels in the range 3.5-5.5 mmol/L. In hyperinsulinemic hypoglycemia (HH), this precise regulation of insulin secretion is perturbed so that insulin continues to be secreted in the presence of hypoglycemia. HH may be due to genetic causes (congenital) or secondary to certain risk factors....

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Persistent hyperinsulinemic hypoglycemia of infancy.

OBJECTIVE To study the nature and clinical course of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) due to nesidioblastosis. DESIGN Clinical, laboratory and therapeutic evaluation of infants with this disorder and study the outcome. SETTING Hospital born neonates and infants referred from other hospitals. SUBJECTS Thirteen infants from 9 families inclusive of four pairs of sib...

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Therapy for Persistent Hyperinsulinemic Hypoglycemia of Infancy

The neonatal disorder persistent hyperinsulinemic hypoglycemia of infancy (PHHI) arises as the result of mutations in the subunits that form the ATP-sensitive potassium (K ATP ) channel in pancreatic b cells, leading to insulin hypersecretion. Diazoxide (a specific K ATP channel agonist in normal b cells) and somatostatin (octreotide) are the mainstay of medical treatment for the condition. To ...

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[Persistent hyperinsulinemic hypoglycemia of infancy: case report]

OBJECTIVE: To report a case of Persistent Hyperinsulinemic Hypoglycemia in twins which is a situation not yet reported in the literature. METHODS: Report of seizures in identical twins, from consanguineous parents, with persistent hypoglycemia as cause of the seizures. Laboratory tests, performed for etiological investigation of the hypoglycemia, included thyroid hormones (T4/TSH), insulin, cor...

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The focal form of persistent hyperinsulinemic hypoglycemia of infancy.

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a disorder characterized by unregulated insulin release, leading to profound hypoglycemia with a major risk of brain damage if not recognized early. The occurrence of PHHI is low in the Western world (~1/50,000 live births), but it can be as high as 1/2,500 live births in communities with high consanguinity. PHHI may be differentiate...

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ژورنال

عنوان ژورنال: Frontiers in Endocrinology

سال: 2016

ISSN: 1664-2392

DOI: 10.3389/fendo.2016.00029